Journal of Pathology and Translational Medicine

Review

Welcoming the new, revisiting the old: a brief glance at cytopathology reporting systems for lung, pancreas, and thyroid: Rita Luis, Balamurugan Thirunavukkarasu, Deepali Jain, Sule Canberk; J Pathol Transl Med. 2024;58(4):165-173. Published online July 15, 2024; DOI: https://doi.org/10.4132/jptm.2024.06.11

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This review addresses new reporting systems for lung and pancreatobiliary cytopathology as well as the most recent edition of The Bethesda Reporting System for Thyroid Cytopathology. The review spans past, present, and future aspects within the context of the intricate interplay between traditional morphological assessments and cutting-edge molecular diagnostics. For lung and pancreas, the authors discuss the evolution of reporting systems, emphasizing the bridge between past directives and more recent collaborative efforts of the International Academy of Cytology and the World Health Organization in shaping universal reporting systems. The review offers a brief overview of the structure of these novel systems, highlighting their strengths and pinpointing areas that require further refinement. For thyroid, the authors primarily focus on the third edition of The Bethesda System for Reporting Thyroid Cytopathology, also considering the two preceding editions. This review serves as an invaluable resource for cytopathologists, offering a panoramic view of the evolving landscape of cytopathology reporting and pointing out the integrative role of the cytopathologist in an era of rapid diagnostic and therapeutic advancements.

Citations

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WHO Reporting System for Lung Cytopathology: Insights Into the Insufficient/Inadequate/Non‐Diagnostic, Atypical and Suspicious for Malignancy Categories and How to Use Them
Zahra Maleki, Sule Canberk, Andrew Field
Cytopathology.2025; 36(5): 434. CrossRef
Reproducibility of the Bethesda system for reporting thyroid cytopathology (TBSRTC): An observational study of 100 patients
Kishori Moni Panda, Reena Naik, Mohd Ghouse Mohiddin
Indian Journal of Pathology and Oncology.2024; 11(4): 385. CrossRef

Case Study

Malignant potential of neuroendocrine microtumor of the pancreas harboring high-grade transformation: lesson learned from a patient with von Hippel-Lindau syndrome: Jongwon Lee, Kyung Jin Lee, Dae Wook Hwang, Seung-Mo Hong; J Pathol Transl Med. 2024;58(2):91-97. Published online March 13, 2024; DOI: https://doi.org/10.4132/jptm.2024.02.13

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Abstract PDF

Pancreatic neuroendocrine microtumor (PNEMT) is a neuroendocrine tumor (NET) < 0.5 cm in diameter, and it is considered benign. We report a PNEMT with high-grade transformation (HGT). A man in his 60s with von Hippel-Lindau syndrome underwent surgical resection of a NET. A second sub-centimeter nodule with a nodule-in-nodule pattern was discovered. The 0.4 cm outer nodule contained clear columnar cells with round nuclei and indistinct nucleoli, while the 0.1 cm inner nodule had eosinophilic cells with an increased nuclear to cytoplasmic ratio, vesicular nuclei, and prominent nucleoli. Tumor cells in the outer and inner nodules were synaptophysin and chromogranin positive. Only the inner nodule was p53 positive, while the outer nodule was exclusively positive for carbonic anhydrase 9 and vimentin. The Ki-67 labeling indices for the outer and inner nodules were 2.1% (grade 1) and 44.3% (grade 3), respectively. This nodule was determined to be a PNEMT with HGT. Our findings suggest that a PNEMT may not always be benign and can undergo HGT.

Citations

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Decoding Pancreatic Neuroendocrine Tumors: Molecular Profiles, Biomarkers, and Pathways to Personalized Therapy
Linda Galasso, Federica Vitale, Gabriele Giansanti, Giorgio Esposto, Raffaele Borriello, Irene Mignini, Alberto Nicoletti, Lorenzo Zileri Dal Verme, Antonio Gasbarrini, Maria Elena Ainora, Maria Assunta Zocco
International Journal of Molecular Sciences.2025; 26(16): 7814. CrossRef
Pancreatic neuroendocrine microtumors in the elderly: A retrospective study using cadaveric pancreatic tissue
Ting Yang, Ke Ren, Xiang-Quan Chen, Taku Toriumi, Yutaro Natsuyama, Jun Li, Aoi Sukeda, Toshitaka Nagao, Shuang-Qin Yi
World Journal of Gastrointestinal Oncology.2025;[Epub] CrossRef
Molecular Basis of Pancreatic Neuroendocrine Tumors
Alesia Maluchenko, Denis Maksimov, Zoia Antysheva, Julia Krupinova, Ekaterina Avsievich, Olga Glazova, Natalia Bodunova, Nikolay Karnaukhov, Ilia Feidorov, Diana Salimgereeva, Mark Voloshin, Pavel Volchkov
International Journal of Molecular Sciences.2024; 25(20): 11017. CrossRef

Original Articles

Lymph node size and its association with nodal metastasis in ductal adenocarcinoma of the pancreas: Jaehoon Shin, Seungbeom Shin, Jae Hoon Lee, Ki Byung Song, Dae Wook Hwang, Hyoung Jung Kim, Jae Ho Byun, HyungJun Cho, Song Cheol Kim, Seung-Mo Hong; J Pathol Transl Med. 2020;54(5):387-395. Published online July 21, 2020; DOI: https://doi.org/10.4132/jptm.2020.06.23

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Abstract PDF

Background
Although lymph node metastasis is a poor prognostic factor in patients with pancreatic ductal adenocarcinoma (PDAC), our understanding of lymph node size in association with PDAC is limited. Increased nodal size in preoperative imaging has been used to detect node metastasis. We evaluated whether lymph node size can be used as a surrogate preoperative marker of lymph node metastasis.
Methods
We assessed nodal size and compared it to the nodal metastatic status of 200 patients with surgically resected PDAC. The size of all lymph nodes and metastatic nodal foci were measured along the long and short axis, and the relationships between nodal size and metastatic status were compared at six cutoff points.
Results
A total of 4,525 lymph nodes were examined, 9.1% of which were metastatic. The mean size of the metastatic nodes (long axis, 6.9±5.0 mm; short axis, 4.3±3.1 mm) was significantly larger than that of the non-metastatic nodes (long axis, 5.0±4.0 mm; short axis, 3.0±2.0 mm; all p<.001). Using a 10 mm cutoff, the sensitivity, specificity, positive predictive value, overall accuracy, and area under curve was 24.8%, 88.0%, 17.1%, 82.3%, and 0.60 for the long axis and 7.0%, 99.0%, 40.3%, 90.6%, and 0.61 for the short axis, respectively.
Conclusions
The metastatic nodes are larger than the non-metastatic nodes in PDAC patients. However, the difference in nodal size was too small to be identified with preoperative imaging. The performance of preoperative radiologic imaging to predict lymph nodal metastasis was not good. Therefore, nodal size cannot be used a surrogate preoperative marker of lymph node metastasis.

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Advances in Radiomics for the Diagnosis and Treatment of Pancreatic Ductal Adenocarcinoma
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Preoperative MRI and CA19-9 for predicting occult lymph node metastasis in small pancreatic ductal adenocarcinoma (≤ 2 cm)
Qiying Tang, Lei Li, Zhiwei Pan, Jianbo Li, Xiaolan Huang, Mengsu Zeng, Haitao Sun, Jianjun Zhou
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Ziyang Wang, Bo Dai, Jian Zhang, Yang Wu, Yunlong Li, Ying Cao, Qi You, Wei Wang, Sunil Singhal, Shuming Nie, Christopher J. Butch, Huiming Cai, Yiqing Wang
Med-X.2025;[Epub] CrossRef
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Ali Kosari, Shokouh Taghipour Zahir, Saadat Eslami
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Long‐term outcomes of neoadjuvant gemcitabine, nab‐paclitaxel, and S1 (GAS) in borderline resectable pancreatic cancer with arterial contact: Results from a phase II trial
Kenichiro Uemura, Naru Kondo, Takeshi Sudo, Tatsuaki Sumiyoshi, Ryuta Shintakuya, Kenjiro Okada, Kenta Baba, Takumi Harada, Yoshiaki Murakami, Shinya Takahashi
Journal of Hepato-Biliary-Pancreatic Sciences.2024; 31(5): 351. CrossRef
Comparison of MRI and CT-based radiomics for preoperative prediction of lymph node metastasis in pancreatic ductal adenocarcinoma
Piaoe Zeng, Chao Qu, Jianfang Liu, Jingjing Cui, Xiaoming Liu, Dianrong Xiu, Huishu Yuan
Acta Radiologica.2023; 64(7): 2221. CrossRef
Prevalence of Adenopathy at Chest Computed Tomography After Vaccination for Severe Acute Respiratory Syndrome Coronavirus 2
Georgeann McGuinness, Jeffrey B. Alpert, Geraldine Brusca-Augello, Lea Azour, Jane P. Ko, Farah Tamizuddin, Elliott K. Gozansky, William H. Moore
Journal of Computer Assisted Tomography.2023; 47(1): 50. CrossRef
Predictive role of radiomics features extracted from preoperative cross-sectional imaging of pancreatic ductal adenocarcinoma in detecting lymph node metastasis: a systemic review and meta-analysis
Mohammad Mirza-Aghazadeh-Attari, Seyedeh Panid Madani, Haneyeh Shahbazian, Golnoosh Ansari, Alireza Mohseni, Ali Borhani, Shadi Afyouni, Ihab R. Kamel
Abdominal Radiology.2023; 48(8): 2570. CrossRef
Regional lymph node metastasis detected on preoperative CT and/or FDG-PET may predict early recurrence of pancreatic adenocarcinoma after curative resection
Ja Kyung Yoon, Mi-Suk Park, Seung-Seob Kim, Kyunghwa Han, Hee Seung Lee, Seungmin Bang, Ho Kyoung Hwang, Sang Hyun Hwang, Mijin Yun, Myeong-Jin Kim
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Alessandro Coppola, Vincenzo La Vaccara, Tommaso Farolfi, Michele Fiore, Roberto Cammarata, Sara Ramella, Roberto Coppola, Damiano Caputo
Biomedicines.2022; 10(9): 2091. CrossRef
Evaluation of the 8th Edition AJCC Staging System for the Clinical Staging of Pancreatic Cancer
Huapyong Kang, Seung-seob Kim, Min Je Sung, Jung Hyun Jo, Hee Seung Lee, Moon Jae Chung, Jeong Youp Park, Seung Woo Park, Si Young Song, Mi-Suk Park, Seungmin Bang
Cancers.2022; 14(19): 4672. CrossRef
Does direct invasion of peripancreatic lymph nodes impact survival in patients with pancreatic ductal adenocarcinoma? A retrospective dual-center study
Daisuke Hashimoto, Sohei Satoi, Mitsuaki Ishida, Kenji Nakagawa, Masaya Kotsuka, Tadataka Takagi, Hironori Ryota, Taichi Terai, Tatsuma Sakaguchi, Minako Nagai, So Yamaki, Takahiro Akahori, Tomohisa Yamamoto, Mitsugu Sekimoto, Masayuki Sho
Pancreatology.2021; 21(5): 884. CrossRef
CA19.9 Serum Level Predicts Lymph-Nodes Status in Resectable Pancreatic Ductal Adenocarcinoma: A Retrospective Single-Center Analysis
Alessandro Coppola, Vincenzo La Vaccara, Michele Fiore, Tommaso Farolfi, Sara Ramella, Silvia Angeletti, Roberto Coppola, Damiano Caputo
Frontiers in Oncology.2021;[Epub] CrossRef

Sarcoma metastasis to the pancreas: experience at a single institution: Miseon Lee, Joon Seon Song, Seung-Mo Hong, Se Jin Jang, Jihun Kim, Ki Byung Song, Jae Hoon Lee, Kyung-Ja Cho; J Pathol Transl Med. 2020;54(3):220-227. Published online April 22, 2020; DOI: https://doi.org/10.4132/jptm.2020.03.04

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Abstract PDF

Background
Reports of metastatic sarcoma to the pancreas are limited. We reviewed the clinicopathologic characteristics of such cases.
Methods
We reviewed 124 cases of metastatic tumors to the pancreas diagnosed at Asan Medical Center between 2000 and 2017.
Results
Metastatic tumors to the pancreas consisted of 111 carcinomas (89.5%), 12 sarcomas (9.6%), and one melanoma (0.8%). Primary sarcoma sites were bone (n = 4); brain, lung, and soft tissue (n = 2 for each); and the uterus and pulmonary vein (n = 1 for each). Pathologically, the 12 sarcomas comprised 2 World Health Organization grade III solitary fibrous tumors/hemangiopericytomas, and one case each of synovial sarcoma, malignant solitary fibrous tumor, undifferentiated pleomorphic sarcoma, osteosarcoma, mesenchymal chondrosarcoma, intimal sarcoma, myxofibrosarcoma, myxoid liposarcoma, rhabdomyosarcoma, subtype uncertain, and high-grade spindle-cell sarcoma of uncertain type. The median interval between primary cancer diagnosis and pancreatic metastasis was 28.5 months. One case manifested as a solitary pancreatic osteosarcoma metastasis 15 months prior to detection of osteosarcoma in the femur and was initially misdiagnosed as sarcomatoid carcinoma of the pancreas.
Conclusions
The metastatic sarcoma should remain a differential diagnosis when spindle-cell malignancy is found in the pancreas, even for solitary lesions or in patients without prior history.

Citations

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Metastatic synovial sarcoma masquerading as primary neuroendocrine tumor of pancreas: a diagnostic conundrum
Sherrin Jacob, Balamurugan Thirunavukkarasu, Rajni Yadav, Anany Gupta, Samagra Agarwal, Shamim A. Shamim, Sameer Rastogi, Adarsh Barwad, Deepali Jain
Clinical Journal of Gastroenterology.2025; 18(3): 499. CrossRef
Metastatic tumors to the pancreas: An institutional experience
Matthew Romanish, Rana Naous
Annals of Diagnostic Pathology.2025; 79: 152528. CrossRef
Visceral Metastases of Osteosarcoma in the Hepatopancreatobiliary System
Anna Hohensteiner, Lars Kowalscheck, Kevin Döring, Gerhard Martin Hobusch, Raphael Johannes Tanios, Oliver Strobel, Reinhard Windhager, Philipp Theodor Funovics
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Vittorio Gebbia, Carlo Carnaghi
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Kodai ABE, Yasutomo SEKIDO, Yasuo KABESHIMA
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Daniel Aparicio-López, Jorge Chóliz-Ezquerro, Carlos Hörndler-Algárate, Mario Serradilla-Martín
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Pancreatic metastasis from sarcoma, an infrequent finding
Daniel Aparicio-López, Jorge Chóliz-Ezquerro, Carlos Hörndler-Algárate, Mario Serradilla-Martín
Gastroenterología y Hepatología (English Edition).2023; 46(5): 376. CrossRef
Acute pancreatitis secondary to osteosarcoma metastasis
Pablo Salmón Olavarría, Ana Gordo Ortega, Maren Eizagirre Ubegun, Verónica Ubieto Capella, Elena Carracedo Vega, Juan Carrascosa Gil, David Ruiz-Clavijo García
Revista Española de Enfermedades Digestivas.2023;[Epub] CrossRef
First Recurrence of Synovial Sarcoma Presenting With Solitary Pancreatic Mass
Raja R Narayan, Greg W Charville, Daniel Delitto, Kristen N Ganjoo
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Levent F Umur, Selami Cakmak, Mehmet Isyar, Hamdi Tokoz
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Smaranda Diaconescu, Georgiana Emmanuela Gîlcă-Blanariu, Silvia Poamaneagra, Otilia Marginean, Gabriela Paduraru, Gabriela Stefanescu
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Panagiotis Dorovinis, Nikolaos Machairas, Stylianos Kykalos, Paraskevas Stamopoulos, George Agrogiannis, Nikolaos Nikiteas, Georgios C. Sotiropoulos
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Case Study

Coexisting Mucinous Cystic Neoplasm of the Pancreas and Type 1 Autoimmune Pancreatitis: Mee-Jeong Kim, Tae Jun Song, Hyoung Jung Kim, Song-Cheol Kim, Myung-Hwan Kim, Seung-Mo Hong; J Pathol Transl Med. 2019;53(2):125-128. Published online November 14, 2018; DOI: https://doi.org/10.4132/jptm.2018.10.25

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Abstract PDF

Type 1 autoimmune pancreatitis (AIP1) is an IgG4-related systemic disease that mimics tumors. We report a rare case of AIP1 accompanied by mucinous cystic neoplasm (MCN). A pancreatic lesion was incidentally detected in a woman in her 60s. After 6 years of follow-up, the lesion abruptly increased in size. Computed tomography showed a 3.5 cm unilocular cyst in the tail of the pancreas and distal pancreatectomy was performed. On microscopic examination, the cyst was lined by mucinous and non-mucinous epithelial cells with mild cytologic atypia. The surrounding stroma comprised ovarian-type spindle cells with progesterone receptor positivity. The pericystic pancreas exhibited multifocal lymphoid follicles, lymphoplasmacytic infiltrations, obliterative phlebitis, and storiform fibrosis. IgG4-positive plasma cell infiltration (215 cells high-power field) and the IgG4/IgG ratio (57%) were increased. Cases of MCN coexisting with AIP1 are extremely rare; only two such cases have been reported in the English-language literature. This third case featured low-grade MCN with AIP1.

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Faridul Haq, Gyeongsin Park, Sora Jeon, Mitsuyoshi Hirokawa, Chan Kwon Jung
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Histological features of autoimmune pancreatitis and IgG4-related sclerosing cholangitis with a correlation with imaging findings
Kenji NOTOHARA
Choonpa Igaku.2023; 50(1): 55. CrossRef
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Bin-Bin Zhang, Xin-Meng Hou, Yu-Qi Chen, Jian-Wei Huo, Er-Hu Jin
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Kenji Notohara
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Mucinous cystic neoplasm of the pancreas with type-1 autoimmune pancreatitis-like lesion
Kevin Gowing, David F. Schaeffer, Hui-Min Yang
Human Pathology: Case Reports.2019; 18: 200339. CrossRef

Original Article

Loss of Progesterone Receptor Expression Is an Early Tumorigenesis Event Associated with Tumor Progression and Shorter Survival in Pancreatic Neuroendocrine Tumor Patients: Sung Joo Kim, Soyeon An, Jae Hoon Lee, Joo Young Kim, Ki-Byung Song, Dae Wook Hwang, Song Cheol Kim, Eunsil Yu, Seung-Mo Hong; J Pathol Transl Med. 2017;51(4):388-395. Published online June 8, 2017; DOI: https://doi.org/10.4132/jptm.2017.03.19

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Abstract PDF

Background
Pancreatic neuroendocrine tumors (PanNETs) are the second most common pancreatic neoplasms and there is no well-elucidated biomarker to stratify their detection and prognosis. Previous studies have reported that progesterone receptor (PR) expression status was associated with poorer survival in PanNET patients.
Methods
To validate previous studies, PR protein expression was assessed in 21 neuroendocrine microadenomas and 277 PanNETs and compared with clinicopathologic factors including patient survival.
Results
PR expression was gradually decreased from normal islets (49/49 cases, 100%) to neuroendocrine microadenoma (14/21, 66.6%) to PanNETs (60/277, 21.3%; p < .001). PanNETs with loss of PR expression were associated with increased tumor size (p < .001), World Health Organization grade (p = .001), pT classification (p < .001), perineural invasion (p = .028), lymph node metastasis (p = .004), activation of alternative lengthening of telomeres (p = .005), other peptide hormonal expression (p < .001) and ATRX/DAXX expression (p = .015). PanNET patients with loss of PR expression (5-year survival rate, 64.1%) had significantly poorer recurrence-free survival outcomes than those with intact PR expression (90%) by univariate (p = .012) but not multivariate analyses. Similarly, PanNET patients with PR expression loss (5-year survival rate, 76%) had significantly poorer overall survival by univariate (p = .015) but not multivariate analyses.
Conclusions
Loss of PR expression was noted in neuroendocrine microadenomas and was observed in the majority of PanNETs. This was associated with increased grade, tumor size, and advanced pT and pN classification; and was correlated with decreased patient survival time by univariate but not multivariate analyses. Loss of PR expression can provide additional information on shorter disease-free survival in PanNET patients.

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Incidence and Prognostic Implications of Lymphovascular Invasion in Node‐Negative Pancreatic Neuroendocrine Tumors: Results From the US Neuroendocrine Study Group
Kota Sahara, Diamantis I. Tsilimigras, Yuki Homma, Jun Kawashima, Shishir K. Maithel, Flavio Rocha, Sharon Weber, Ryan Fields, Kamran Idrees, George A. Poultsides, Cliff Cho, Itaru Endo, Timothy M. Pawlik
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Mohamed Mortagy, Marie Line El Asmar, Kandiah Chandrakumaran, John Ramage
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Review

Cytology Specimen Management, Triage and Standardized Reporting of Fine Needle Aspiration Biopsies of the Pancreas: Won Jae Yoon, Martha Bishop Pitman; J Pathol Transl Med. 2015;49(5):364-372. Published online August 10, 2015; DOI: https://doi.org/10.4132/jptm.2015.07.19

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Abstract PDF

The recent advances in pancreas cytology specimen sampling methods have enabled a specific cytologic diagnosis in most cases. Proper triage and processing of the cytologic specimen is pivotal in making a diagnosis due to the need for ancillary testing in addition to cytological evaluation, which is especially true in the diagnosis of pancreatic cysts. Newly proposed terminology for pancreaticobiliary cytology offers a standardized language for reporting that aims to improve communication among patient caregivers and provide for increased flexibility in patient management. This review focuses on these updates in pancreas cytology for the optimal evaluation of solid and cystic lesions of the pancreas.

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Original Articles

Diagnostic Accuracy of Endoscopic Ultrasound-Guided Fine Needle Aspiration Cytology of Pancreatic Lesions: Hae Woon Baek, Min Jee Park, Ye-Young Rhee, Kyoung Bun Lee, Min A Kim, In Ae Park; J Pathol Transl Med. 2015;49(1):52-60. Published online January 15, 2015; DOI: https://doi.org/10.4132/jptm.2014.10.26

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Abstract PDF

Background
Endoscopic ultrasound–guided fine needle aspiration cytology (EUS-FNAC) is currently the most commonly used procedure for obtaining cytologic specimens of the pancreas. It is accurate, minimally invasive, safe and cost-effective. However, there is discrepancy between cytological and surgical diagnoses. This study was aimed at evaluating the diagnostic accuracy of EUS-FNAC of the pancreas. Methods: We performed a retrospective review of 191 cases of pancreatic lesions initially diagnosed by EUS-FNAC with subsequent histological diagnosis between 2010 and 2012 in the Department of Pathology, Seoul National University Hospital. Cytologic and surgical diagnoses were categorized into five groups: negative, benign, atypical, malignant, and insufficient for diagnosis. Subsequently, 167 cases with satisfactory yield in both surgical and cytology specimens were statistically analyzed to determine correlations with diagnosis. Results: In comparison to surgical diagnoses, cytologic diagnoses were true-positive in 103 cases (61.7%), true-negative in 28 cases (16.8%), false-positive in 9 cases (5.4%), and false-negative in 27 cases (16.1%). The diagnostic accuracy was 78.4%, sensitivity was 79.2%, and specificity was 75.7%. The positive predictive value was 92.0%, and negative predictive value was 50.9%. Conclusions: EUS-FNAC has high accuracy, sensitivity, specificity and positive predictive value. Overcoming the limitations of EUS-FNAC will make it a useful and reliable diagnostic tool for accurate evaluation of pancreatic lesions.

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Clinical and Prognostic Significances of Cytokeratin 19 and KIT Expression in Surgically Resectable Pancreatic Neuroendocrine Tumors: Eun-Mi Son, Joo Young Kim, Soyeon An, Ki-Byung Song, Song Cheol Kim, Eunsil Yu, Seung-Mo Hong; J Pathol Transl Med. 2015;49(1):30-36. Published online January 15, 2015; DOI: https://doi.org/10.4132/jptm.2014.10.23

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Abstract PDF

Background
Pancreatic neuroendocrine tumors (PanNETs) are malignant endocrine neoplasms that present diverse clinical behaviors. Therefore, identification of biomarkers of PanNETs is important for stratification of the prognosis of PanNET patients. Recently, cytokeratin 19 (CK19) and KIT expression were reported to have prognostic significance in PanNET patients. Methods: To identify their prognostic significance, CK19 and KIT protein expression were assessed in 182 surgically resected PanNETs and compared with clinicopathologic factors. Results: Of 182 PanNETs cases, CK19 and KIT expression was noted in 97 (53.3%) and 16 (8.8%) cases, respectively. PanNET patients with CK19 expression had larger tumors (p=.006), higher World Health Organization (WHO) grade (p=.002) and pT classification (p<.001), increased distant metastasis (p=.004), and lymphovascular (p=.012) and perineural (p=.019) invasion. Similarly, those with KIT expression had larger tumors (p=.030), higher WHO grade (p=.001), advanced pT classification (p<.001), distant metastasis (p=.001), and lymphovascular invasion (p=.014). The 5-year survival rate for PanNET patients with KIT expression was significantly lower (62%) than that of patients without KIT expression (77%, p=.011), as determined by univariate but not by multivariate analyses. Conclusions: CK19 and KIT expression correlate with higher metastatic potential and advanced disease stage, and KIT expression is associated with worse survival in PanNET patients.

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Brief Case Report

Pulmonary Hodgkin Lymphoma in a Patient with Crohn’s Disease: Jae-Young Park, Juhie Lee; Korean J Pathol. 2014;48(5):387-389. Published online October 27, 2014; DOI: https://doi.org/10.4132/KoreanJPathol.2014.48.5.387

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Review & Perspective

Acinar Cell Carcinoma of the Pancreas: Clinical and Cytomorphologic Characteristics: Adam D. Toll, Ralph H. Hruban, Syed Z. Ali; Korean J Pathol. 2013;47(2):93-99. Published online April 24, 2013; DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.2.93

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Abstract PDF

Acinar cell carcinoma is a rare malignant epithelial neoplasm with predominantly exocrine acinar differentiation and is seen primarily in older men (mean age, 62 years). The presenting symptoms are usually non-specific, and jaundice is often not present. Symptoms relating to the overproduction and release of lipase into the circulation are present in 10-15% of patients. Characteristic cytomorphologic features include a population of cells with minimal pleomorphism, eccentrically placed nuclei with a single prominent nucleoli and moderate hyperchromasia. The cytoplasm is finely granular, and the background may contain granular debris secondary to cytolysis. A significant proportion of the cases also have a minor neuroendocrine component or scattered neuroendocrine cells. Approximately 50% of patients have metastatic disease at presentation, often restricted to the regional lymph nodes and liver. The prognosis is poor, only slightly better than that of pancreatic ductal adenocarcinoma.

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DNA Mismatch Repair Abnormalities in Acinar Cell Carcinoma of the Pancreas
Weiguo Liu, Jinru Shia, Mithat Gönen, Maeve A. Lowery, Eileen M. O’Reilly, David S. Klimstra
Pancreas.2014; 43(8): 1264. CrossRef
Tumeurs acinaires du pancréas : particularités pronostiques. A propos de 4 cas
Z. Mzoughi, N. Azzabou, N. Miloudi, L. Gharbi, M. T. Khalfallah
Journal Africain d'Hépato-Gastroentérologie.2014; 8(3): 113. CrossRef

Case Report

Adenocarcinoma with Intraductal Papillary Mucinous Neoplasm Arising in Jejunal Heterotopic Pancreas: Ju Young Song, Jee Young Han, Sun Keun Choi, Lucia Kim, Suk Jin Choi, In Suh Park, Young Chae Chu, Kyu Ho Kim, Joon Mee Kim; Korean J Pathol. 2012;46(1):96-100. Published online February 23, 2012; DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.1.96

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Abstract PDF

A 74-year-old man suffered from jejunal perforation and adhesion to sigmoid colon due to adenocarcinoma associated with intraductal papillary mucinous neoplasm (IPMN) arising in a jejunal heterotopic pancreas. The jejunal lesion showed direct extension to the sigmoid colon, which was mistaken as sigmoid colon cancer by surgeons. Malignant transformation is a rare complication of a heterotopic pancreas. About half of malignancies in reported cases were ductal adenocarcinoma arising in the stomach, and the jejunal location is extremely rare. Furthermore, IPMN is also uncommon finding in a heterotopic pancreas.

Citations

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Ya-Xin Wang, Jing Wang, Shi-Xiu Liang, Qi Wu
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Pancreatic Intraductal Papillary Mucinous Neoplasm Arising Within Heterotopic Pancreas Tissue of the Stomach
Rinrada Worapongpaiboon, Kasenee Tiankanon, Benjamin L. Mazer, Saowanee Ngamruengphong
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Intraductal Papillary Mucinous Neoplasm Arising from Heterotopic Pancreas in Stomach: A Case Report and Review of Literature
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International Journal of Surgical Pathology.2023; 31(5): 708. CrossRef
Clinicopathological features of intraductal papillary mucinous neoplasm derived from ectopic pancreas: A systematic review
Jiro Kimura, Takehiro Okabayashi, Kenta Sui, Takahiro Murokawa, Motoyasu Tabuchi, Masaki Aida, Jun Iwata, Yasuhiro Hata
Surgery Open Science.2022; 8: 62. CrossRef
Clinicopathological Features of Intraductal Papillary Mucinous Neoplasm Derived from Ectopic Pancreas: A Systematic Review
Jiro Kimura, Takehiro Okabayashi, Kenta Sui, Takahiro Murokawa, Motoyasu Tabuchi, Masaki Aida, Jun Iwata, Yasuhiro Hata
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Naoko Nambu, Takashi Yamasaki, Nami Nakagomi, Tsutomu Kumamoto, Tatsuro Nakamura, Akio Tamura, Toshihiko Tomita, Hiroto Miwa, Hisashi Shinohara, Seiichi Hirota
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Intraductal Papillary Mucinous Neoplasm Arising in a Heterotopic Pancreas Treated by Percutaneous Endoscopic Intragastric Surgery
Shoko Moue, Yoshimasa Akashi, Koichi Ogawa, Katsuji Hisakura, Tsuyoshi Enomoto, Yusuke Ohara, Yohei Owada, Shingo Sakashita, Tatsuya Oda
The Japanese Journal of Gastroenterological Surgery.2020; 53(4): 371. CrossRef
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Original Article

Fine Needle Aspiration Cytology of Solid and papillary Neoplasm of the Pancreas: Report of a Case.: Mee Yon Cho, Kwang Gil Lee, Kyi Beom Lee, Hyeun Joo Jeong, Woo Hee Jung; J Pathol Transl Med. 1990;1(1):85-92.

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Abstract PDF: We present the cytologic features of a case of solid and papillary neoplasm of the pancreas. Cytologically, the tumor was composed of a monotonous population of polygonal cells containing ecentrically located round nuclei with one or two distinct small nucleoli and a finely stippled chromatin pattern. The tumor cells were similar to those of the islet cell tumor and showed isolated loosety aggregated and solid sheedts or large cell clumps. The large cell clumps revealed a branching papillary structure containing fibrovascular central core, which is characteristic histologic feature of solid and papillary neoplasm of the pancreas. The case was confirmed by tissue examination including histochemical immunohistochemical and electron microscopical studies. Utrastructurally, the tumor cells contanined a few membrane-bound electron dense granules.

Case Report

Simultaneous Pancreatic Serous Microcystic Adenoma and Intraductal Papillary Mucinous Tumor of the Pancreas: A Case Report.: Hyoung Jong Kwak, Young Kon Kim, Baik Hwan Cho, Woo Sung Moon; Korean J Pathol. 2011;45:S29-S31.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.S1.S29

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Abstract PDF: Serous cystadenomas of the pancreas account for approximately a third of pancreatic cystic neoplasms. Their coexistence with a second tumor is extremely rare. We now report a case of a serous microcystic adenoma combined with an intraductal papillary mucinous tumor of the pancreas in a 69-year-old man. Abdominal computed tomography scans demonstrated an incidental cystic mass in the body with cystic dilatation of the duct in the head of the pancreas. Central pancreatectomy with pancreatico-jejunostomy, and cyst excision of the pancreatic head were performed. Histologic examination demonstrated a serous microcystic cystadenoma in the body coexisting with an intraductal papillary mucinous adenoma in the head of the pancreas. This case study highlights the importance of careful intra-operative and pathologic examination for synchronous pancreatic tumors.

Original Article

Clinical Outcome of Surgically Resected Pancreatic Intraductal Papillary Mucinous Neoplasm According to the Marginal Status: A Single Center Experience.: Sun A Kim, Eunsil Yu, Song Cheol Kim, Jihun Kim; Korean J Pathol. 2010;44(4):410-419.; DOI: https://doi.org/10.4132/KoreanJPathol.2010.44.4.410

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Abstract PDF

BACKGROUND
Surgical resection is the treatment of choice of intraductal papillary mucinous neoplasm (IPMN) of the pancreas. However, the benefit of clearing resection margin is still controversial.
METHODS
We reviewed 281 surgically resected cases of IPMN. The recurrences were compared according to the histologic grade (benign or borderline IPMN, malignant noninvasive IPMN, invasive carcinoma) and size (pancreatic intraepithelial neoplasia, PanIN, less than 0.5 cm in the long axis; and IPMN, greater than or equal to 0.5 cm) of the residual lesions at the resection margin.
RESULTS
Sixty cases (21.4%) were invasive carcinoma, and 221 (78.6%) noninvasive cases included 87 (31.0%) benign, 107 (38.1%) borderline and 11 (3.9%) malignant noninvasive IPMN cases. In noninvasive IPMN, increased recurrence in patients with five or more years of follow-up was only related to the involvement of resection margin by severe dysplasia. The recurrence of invasive carcinoma was high (27.3%) even when the resection margin was clear, and was not related to the grade or size of residual tumors at the resection margin.
CONCLUSIONS
Invasiveness is a strong risk factor for recurrence in IPMN regardless of the status of the resection margin. However, in noninvasive IPMN, histologic grading of residual lesions at the resection margin predicts local recurrence.

Citations

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Systematic review of challenging issues in pathology of intraductal papillary mucinous neoplasms
Laura D. Wood, N. Volkan Adsay, Olca Basturk, Lodewijk A.A. Brosens, Noriyoshi Fukushima, Seung-Mo Hong, Sung-Joo Kim, Jae W. Lee, Claudio Luchini, Michaël Noë, Martha B. Pitman, Aldo Scarpa, Aatur D. Singhi, Mariko Tanaka, Toru Furukawa
Pancreatology.2023; 23(7): 878. CrossRef
The Use of Intraoperative Frozen Sections in Guiding the Extent of Pancreatic Resections for Intraductal Papillary Mucinous Neoplasms
Zhikai Chi, Deepti Dhall, Richard Mertens
Pancreas.2022; 51(1): 63. CrossRef
Recurrence of non-invasive intraductal papillary municious neoplasm seven years following total pancreatectomy
Nayima M. Clermont Dejean, Sinziana Dumitra, Jeffrey S. Barkun
International Journal of Surgery Case Reports.2013; 4(9): 789. CrossRef

Case Reports

Intraductal Papillary Mucinous Tumor Simultaneously Involving the Liver and Pancreas: A Case Report.: Bong Hee Park, Jae Hee Suh, Hee Jeong Cha, Young Min Kim, Hye Jeong Choi; Korean J Pathol. 2010;44(1):83-86.; DOI: https://doi.org/10.4132/KoreanJPathol.2010.44.1.83

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Abstract PDF

We describe here a 67-year-old man who was diagnosed with a rare case of intraductal papillary mucinous tumors that occurred simultaneously in the liver and pancreas. Abdominal computed tomography showed a tubular and cystic dilatation of the pancreatic duct in the pancreas tail, which suggested an intraductal papillary mucinous tumor (IPMT), and multiple intrahepatic duct stones. The patient underwent a distal pancreatectomy with splenectomy and a lateral segmentectomy of the liver. Microscopic examination showed an intraductal papillary mucinous neoplasms of borderline malignancy in the pancreas and a non-invasive intraductal papillary mucinous tumor with moderate dysplasia of the bile duct. Although several cases of intraductal papillary mucinous neoplasm of the liver (IPNL) without any pancreatic association have been described, the simultaneous presentation of both IPMT of the pancreas and IPNL is very rare. The patient has been doing well for 10 months postoperatively.

Citations

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Surgical resection for simultaneous intraductal papillary mucinous neoplasm of the bile duct and pancreatic duct: A case report
Xiao-Rui Huang, Deng-Sheng Zhu, Ya-Hong Yu
World Journal of Gastrointestinal Surgery.2025;[Epub] CrossRef
Reoperation for heterochronic intraductal papillary mucinous neoplasm of the pancreas after bile duct neoplasm resection: A case report
Gang Xiao, Tao Xia, Yi-Ping Mou, Yu-Cheng Zhou
World Journal of Gastrointestinal Surgery.2023; 15(7): 1542. CrossRef
Intraductal papillary neoplasm of the bile duct: The new frontier of biliary pathology
Federico Mocchegiani, Paolo Vincenzi, Grazia Conte, Daniele Nicolini, Roberta Rossi, Andrea Benedetti Cacciaguerra, Marco Vivarelli
World Journal of Gastroenterology.2023; 29(38): 5361. CrossRef
Multicentric recurrence of intraductal papillary neoplasm of bile duct after spontaneous detachment of primary tumor: A case report
Hiroki Fukuya, Akifumi Kuwano, Shigehiro Nagasawa, Yusuke Morita, Kosuke Tanaka, Masayoshi Yada, Akihide Masumoto, Kenta Motomura
World Journal of Clinical Cases.2022; 10(3): 1000. CrossRef
Co-occurrence of IPMN and malignant IPNB complicated by a pancreatobiliary fistula: A case report and review of the literature
Xu Ren, Chun-Lan Zhu, Xu-Fu Qin, Hong Jiang, Tian Xia, Yong-Ping Qu
World Journal of Clinical Cases.2019; 7(1): 102. CrossRef
Synchronous pancreatic adenocarcinoma and intrahepatic cholangiocarcinoma arising in the context of intraductal papillary neoplasms
Anmol Bansal, Swan N. Thung, Hongfa Zhu, Myron Schwartz, Sara Lewis
Clinical Imaging.2016; 40(5): 897. CrossRef

A Case of Pancreatitis Presenting with Pancreatic Panniculitis: A Case Report.: Seong Hyun Kim, Sun Hee Chang, Hai Jin Park; Korean J Pathol. 2009;43(6):566-569.; DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.6.566

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Abstract PDF

Pancreatic panniculitis is a rare disease in which necrosis of fat in the panniculus and other distant foci occur in the setting of pancreatic disease. The most common pancreatic disorders associated with pancreatic panniculitis are acute and chronic pancreatitis, especially the alcohol-related types and pancreatic carcinoma. We now report a case of pancreatic panniculitis in which skin eruptions, not abdominal symptoms, were the presenting symptom. A 45-year-old man presented with multiple erythematous tender subcutaneous nodules on the shins, arthralgia of both ankles and swelling of the left foot. He was diagnosed as having pancreatitis 15 months ago. Histopathologic findings of a skin biopsy showed focal fat necrosis with anucleated "ghost-like" fat cells with a thick shadowy wall. Laboratory evaluation revealed an increase in serum amylase and lipase. We diagnosed this patient's problem as pancreatic panniculitis associated with a relapse of pancreatitis.

Citations

Citations to this article as recorded by

Pancreatic Panniculitis in Patients with Chronic Pancreatitis: Case Report and Review of Literature
Eui Joong Kim, Min Su Chu, Ki Chang Sohn, Dong Ho Cho, Ga Hye Na, Haak Cheoul Kim, Eun Young Cho
The Korean Journal of Gastroenterology.2017; 69(1): 83. CrossRef

Original Articles

Clear Cell Islet Cell Tumor of the Pancreas: An Immunohistochemical and Ultrastructural study.: Seung Sam Paik, Young Ha Oh, Eun Kyung Hong, Moon Hyang Park, Jung Dal Lee; Korean J Pathol. 1997;31(2):162-166.

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Abstract PDF: A clear cell islet cell tumor of the pancreas is extremely rare and characterized by extensive clear cell components. Electron microscopic and immunohistochemical findings are essential to prove that the mass with clear cells is an unusual manifestation of an islet cell tumor. Herein, we report a case of clear cell islet cell tumor of a 54-year-old woman with abdominal pain. The tumor was composed of polygonal clear cells arranged in nests, trabeculae, and ribbon pattern with the extensively fibrous stroma. These tumor cells showed strong reactivity for chromogranin and weak reactivity for somatostatin and glucagon. An electron microscope revealed that the important contributing factor of the clear cytoplasmic change was mainly due to an accumulation of lipid droplets, coupled with cytoplasmic swelling in some areas. Some tumor cells showed many endosecretory granules ranging from 111 to 297nm in diameter. In the clinical and immunohistochemical findings these granules were consistent with somatostatin granules in morphology and size.

Expression of Matrix Metalloproteinase-2 (MMP-2) and Tissue Inhibitor of Metalloproteinase-2 (TIMP-2) in Pancreatic Ductal Adenocarcinoma.: Mi Jin Gu, Young Kyung Bae, Joon Hyuk Choi; Korean J Pathol. 2004;38(2):73-78.

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Abstract PDF: BACKGROUND
Matrix metalloproteinase-2 (MMP-2) is known to be one of the key molecules for tumor invasion and metastasis. MMP-2 activity is modulated through interaction with the tissue inhibitor of metalloproteinase-2 (TIMP-2). The purpose of this study was to evaluate the expression of MMP-2 and TIMP-2 in pancreatic ductal adenocarcinoma.
METHODS
Using immunohistochemical staining, we investigated the expression of MMP-2 and TIMP-2 in 30 pancreatic ductal adenocarcinomas and 10 normal pancreas.
RESULTS
MMP-2 expression was present in tumor cells in 11 cases, and in stromal cells in 24 cases, out of 30 carcinomas. MMP-2 expression of tumor cells was significantly higher in poorly differentiated adenocarcinomas than in well/moderately differentiated adenocarcinomas, and in cases with vascular invasion than in cases without. MMP-2 expression was stronger in the marginal areas than in the central area of the tumor. TIMP-2 expression was detected in the tumor and stromal cells of all carcinomas. MMP-2 and TIMP-2 expression had no significant correlation with tumor size, lymph node metastasis, or TNM stage. MMP-2 expression was not correlated with TIMP-2 expression.
CONCLUSIONS
These results suggest that MMP-2 expression may play an important role in the invasive property of pancreatic ductal adenocarcinoma, whereas TIMP-2 expression increases as a reaction to invasion.

Ultrastructural and Immunohistochemical Investigations of Exocrine and Endocrine Cells in Fetal Human Pancreas.: Jung Ran Kim, Je G Chi, Jung Hee Cho; Korean J Pathol. 1995;29(3):286-295.

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Abstract PDF: The pancreas consists of two types of tissue arising from same primitive cells, but with entirely different functions. Although the adult human pancreas and fetal islet tissue have been the subject of numerous electron microscopic studies, little is known of the ultrastructure of the developing human exocrine pancreas. The purpose of the current study is to investigate development of endo and exocrine of pancreas, especially during the middle trimester of human fetal life, which is the period of acinar cell maturation. Fresh autopsy specimens of pancreas, taken from 15 human fetuses at the 12th (n=2), 13-16th (n=5), 17-20th (n=4), 21-24th (n=2) and 25-28th (n=2) weeks of gestation, were studied electron microscopically, and immunohistochemically. Antisera against insulin, somatostatin, glucagon, pancreatic polypeptide and gastrin, were used for immunohistochemistry. By the 12th week, primitive exocrine acini were identified and these were matured rapidly in the next 6 weeks. At the 17th week stage, ultrastructural examination revealed atypical zymogen granules in the acinar cells. These became progressively less numerous in the 21-28 week period when classical zymogen granules increased upto the level of adult stage. All the endocrine cells were found at the 12th week, forming primitive or mature islets. The relative ratio of endocrine cells at the 12th week was about 35.4%, 24.9%, 39.8%, 0.5% for A, B, D & PP cell, respectively. But at the 25th to 28th week of development, the relative numbers of A and D cells decreased somewhat, whereas those of the B cells increased. The PP cells were constant. The G cells were found at the 12th week of fetal period, which appeared through out the on period.

Case Report

Mixed Ductal-Endocrine Carcinoma of the Pancreas: A Case Report.: Ok Jun Lee, Yong Mee Cho, Hyang Im Lee, Duck Jong Han, Jae Y Ro; Korean J Pathol. 2004;38(5):353-356.

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Abstract PDF: Mixed ductal-endocrine carcinoma of the pancreas is composed of ductal and endocrine carcinoma components and each component makes up a significant proportion in the primary tumor as well as in the tumor of metastatic sites. Mixed ductal-endocrine pancreatic carcinoma is exceptionally rare and, to our knowledge, only five cases have been reported in the literature. Recently we experienced a case of mixed ductal-endocrine pancreatic carcinoma with regional lymph node and hepatic metastases in a 63-year-old woman. Here, we report a case of mixed ductal-endocrine pancreatic carcinoma with a review of the literature.

Original Article

Solid and Papillary Epithelial Neoplasm of the Pancreas Diagnosed by Percutaneous Fine Needle Aspiration Biopsy.: Chan Kum Park, Jung Dal Lee; Korean J Pathol. 1990;24(3):304-309.

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Abstract PDF: Solid and papillary epithelial neoplasm of the pancreas is a rare, low grade malignant tumor of adolescence. The tumor we described was diagnosed by following preoperative percutaneous fine needle aspiration biopsy and confirmed by tissue examination. Characteristic cytologic findings were monotonous cells arranged in papillary fronds, acinus, and cords in necrotic and hemorrhagic backgrounds. Occasional foamy histiocytes and hyaline globules were present. The cytoplasm of the tumor cells were moderate in amount, pale eosinophilic or vacuolated with distinct borders. The nuclei were centrally located and round. There were one or two small nucleoli and finely granular chromatin throughout the nucleus.

Case Reports

Oncocytic Type Intraductal Papillary Mucinous Neoplasm Mimicking Mucinous Cystic Neoplasm of the Pancreas: A Case Report.: Tae Jung Jang, Jong Im Lee; Korean J Pathol. 2008;42(3):172-174.

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Abstract PDF: Oncocytic type intraductal papillary mucinous neoplasm is a newly defined subgroup of intraductal papillary mucinous neoplasms. A 35-year-old woman presented complaining of epigastric pain for one month. Enhanced computed tomography revealed a multilocular cystic mass in the distal body and tail of the pancreas. Endoscopic retrograde cholangiopancreatography showed no communication between the mass and the main pancreatic duct. The patient underwent a distal pancreatectomy and splenectomy. Microscopical examination showed a fibrous cyst wall; polypoid tumors exhibiting thin stalks, with extensive arborizing papillary growths from these stalks; and a focally cribriform pattern, lined by plump cells with abundant eosinophilic and granular cytoplasm. Red granules were detected in the cytoplasm of tumor cells on modified Gomori trichrome stain. Ultrastructurally, the tumor cells contained abundant cytoplasm packed with numerous mitochondria and intracellular and intercellular lumina. We describe an oncocytic type intraductal papillary mucinous neoplasm having the clinical characteristics of a mucinous cystic neoplasm.

Serous Cystadenoma of the Pancreas: A case report.: Young Kyoung Bae, Woo Young Jang, Kyoung Chan Choi, Joon Hyuk Choi, Won Hee Choi; Korean J Pathol. 1996;30(1):68-71.

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Abstract PDF: Serous cystadenoma of the pancreas, also known as microcystic adenoma or glycogen-rich cystadenoma, is an unusually benign tumor. It is usually large and composed microscopically of many small cysts lined by small, cuboidal or flattened cells containing abundant glycogen. It has been suggested that serous cystadenoma probably arise from the ductular cells or centroacinar cells. Herein, we report on a case of serous cystadenoma of the pancreas in a 55-year-old female. The tumor, measuring 13.5x11.5x10.0 cm, was located in the head of the pancreas and the cut surface revealed a sponge-like appearance due to innumerable tiny cysts containing clear serous fluid. Microscopic analysis showed cystic spaces lined by cuboidal cells with intracytoplasmic glycogen.

Leiomyosarcoma of the Pancreas: A case report.: Bong Kyung Shin, Jung Suk Moon, Hwa Eun Oh, Nam Hee Won, Jong Sang Choi; Korean J Pathol. 1999;33(9):733-736.

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Abstract PDF: Most of the malignant tumors of the pancreas are adenocarcinomas arising from the ductal epithelium. Primary leiomyosarcoma of the pancreas, even though it is the most common sarcoma of the pancreas, is very rare. We present a case of leiomyosarcoma of the pancreas, probably primary, with metastases to the stomach, lymph nodes, and abdominal wall. A 52-year-old woman visited the hospital with vague right upper abdominal pain and weigh loss of 6 kg for 2 months. The radiological and endoscopic examination revealed that she had a large heterogeneous mass, 11 cm in size, in the pancreatic body and tail, a 4 cm-sized mass in the paraaortic area, and a 3 cm-sized polypoid mass in the stomach. Histologically, they were all similar to one another and composed of markedly pleomorphic cells. Immunohistochemical and electron microscopic studies showed definite smooth muscle differentiation of the tumor cells. Two months later, the patient underwent an excision of a new 3 cm mass in the right lower abdominal wall, showing features of well differentiated leiomyosarcoma.

Fine Needle Aspiration Cytology of the Intraductal Papillary Mucinous Tumor of the Pancreas: A Case Report.: In Gu Do, Jae Hoon Park, Youn Wha Kim, Ju Hie Lee, Moon Ho Yang, Sung Wha Hong, Yong Koo Park; J Pathol Transl Med. 2003;14(2):91-95.

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Abstract PDF: Intraductal papillary mucinous tumor of the pancreas is characterized by intraductal papillary proliferation of mucin-producing epithelial cells with or without excessive mucin secretion. According to the degree of epithelial dysplasia, intraductal papillary mucinous tumor is classified into adenoma, borderline tumor, and carcinoma. We recently experienced a case of fine needle aspiration cytology of the intraductal papillary mucinous adenoma in a 69-year-old male. The fine needle aspiration cytology yielded flat sheets of columnar, mucin containing epithelial cells in the background of dense mucin containing degenerated cellular material and histiocytes.

Fine Needle Aspiration Cytology of Mucinous Cystic Carcinoma of the Pancreas: A Case Report.: Kyungji Lee, Ahwon Lee, Kyo Young Lee, Chang Suk Kang, Sang In Shim; J Pathol Transl Med. 2005;16(2):88-92.

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Abstract PDF: Mucious cystic neoplasm of pancreas is a cystic neoplasm composed of columnar, mucin-producing epithelium and is supported by ovarian-type stroma. The key to the cytologic evaluation of pancreatic cystic lesions is to recognize the cytologic components as being diagnostic of a mucin-producing cystic neoplasm, as all of these neoplasms need to be resected. We report the use of fine needle aspiration cytology in the diagnosis of an invasive mucinous cystic carcinoma confirmed by partial pancreatectomy. The cytologic specimen showed a abundant mucin background and sheets or papillae of neoplastic cells. There are mucin-containing columnar cells that show a variable degree of cytologic atypia.

Epithelial Cysts in the Intrapancreatic Accessory Spleen that Clinically Mimic Pancreatic Cystic Tumor: A Report of Two Cases.: Jae Kyung Won, You Jeong Lee, Gyeong Hoon Kang; Korean J Pathol. 2005;39(6):437-441.

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Abstract PDF: Cystic lesions in the accessory spleen are extremely rare and they present a challenging clinical differential diagnosis. We report here on two cases of epithelial cyst of intrapancreatic accessory spleen that mimicked pancreatic cystic tumor. In both cases, the patients underwent distal pancreatectomy under the impression of a benign cystic tumor of the pancreas. Unilocular or multilocular cysts in the pancreas tail were observed, and these were later shown to be epithelial cysts in the accessory spleen located within the pancreatic tail. The cysts were lined by columnar, cuboidal or stratified squamous epithelium.

Somatostatin Secreting Islet Cell Adenoma: A case report.: Tae Sook Kim, Je G Chi; Korean J Pathol. 1996;30(7):630-634.

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Abstract PDF: Somatostatin secreting islet cell adenoma(somatostatinoma) of the pancreas is a rare entity. Less than 30 cases of pancreatic somatostatinoma could be searched in the world literature. We present a case of somatostatinoma of the pancreas in a 64-year-old woman. This patient had suffered from diabetes, cholelithiasis and intermittent diarrhea. Laboratory examination revealed steatorrhea, blood somatostatin level of 30 pg/ml, and fasting blood glucose level of 116 mg/dl. Subtotal pancreatectomy was done after radiological demonstrations of a round tumor mass in the pancreas. It was a 2 cm-sized well demarcated yellowish brown round solid mass located in the proximal pancreas. A retention cyst was seen just distal to the tumor. Histologically, tumor masses consisted of polygonal cells with distinct cell border, having granular eosinophilic cytoplasm and small bland looking nuclei. The tumor cells were arranged in small solid islands and trabeculae, separated by fibrovascular stroma. Immunohistochemical stain for somatostatin was positive in the cytoplasm of the individual tumor cell, and ultrastructurally variable sized membrane bound electron dense granules of 200 nm in average diameter were found in tumor cells.

Original Article

Histologic Variations of Intramural Heterotopic Pancreas in Gastrointestinal Tract Analysis of 15 Cases.: Seung Sook Lee, Yong Il Kim, Woo Ho Kim, Eun Sil Yu; Korean J Pathol. 1991;25(6):520-527.

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Abstract PDF: We reviewed a total of 15 cases of heterotopic pancreatic tissue within the gastrointestinal wall(intramural type), and compared with 3 extramural ones. Intramural heterotopic pancreatic lesions were located in the antrum(33%), pylorus(20%), and body of stomach(7%), and the remainders in the duodenum(40%). Only two cases presented with chinical symptoms by their existence. Two of them were situated within the submucosa, 3 in the muscularis, 6 in submucosa-muscularis, 2 in the muscularis-subserosa, and 1 in the susbmucosa-subserosa. Intramural type was featured with their structural heterogeneity compared to the extramural ones; 10 cases showed participation of gastrointestinal mucosal elements, and some accompanied tissue elements that were indistinguishable from submucosal epithelial heterotopia or microduplication cyst of the stomach. Langerhans islets were found in 67%, and one developed islet cell tumor. The above results suggest that the initially engrafted heterotopic pancreatic tissue becomes modified and presents with heterogeneity of endodermal and mesodermal tissue-derived components by its intramural growth during the gastrointestinal organogenesis; failure of opening of its drainage system into the gastrointestinal lumen may result in the increase of intraductal pressure with subsequent atrophy of the acinar tissue and various metaplastic changes of ductal epithelium, aside from induction of smooth muscle coat around the heterotopic tissue.

Case Report

Intraductal Papillary-Mucinous Neoplasm of the Pancreas: A case report.: Ji Eun Kim, Young Hyeh Ko, Howe Jung Ree, Yong Il Kim, Poong Ryul Lee; Korean J Pathol. 1996;30(8):726-732.

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Abstract PDF: Intraductal papillary mucinous neoplasm (IPMN) is a recently recognized clinicopathologic entity characterized by dilatation of pancreatic duct filled with copious mucin and papillary ductal epithelial proliferation ranging from simple hyperplasia to invasive carcinoma. The exact clinicopathologic identification of this tumor is important because of favorable prognosis contrast to that of conventional ductal adenocarcinoma. Herein we report a case of surgically resected typical IPMN. A 59-year-old man had a long history of diabetes mellitus with epigastric pain of 4 months duration. Ultrasonography and computed tomographic examination revealed cystic dilatations of main pancreatic duct in the head. The patient underwent total pancreatectomy. The gross appearance showed diffuse dilatation of main pancreatic duct associated with cystic dilatation of subbranches in the uncinate process. Histologic examination revealed diffuse papillary proliferations lined by mucinous epithelium with mild atypism within ectatic ducts. No invasive carcinoma was noted. Histochemically, the papillary epithelium contained mostly neutral and acid sialomucin.

Original Article

Immunohistochemical and Ultrastructural Cellular Differentiation in Papillary and Solid Epithelial Neoplasm of the Pancreas.: Jae Hyuck Lee, Min Cheol Lee, Chang Soo Park, Kyu Hyuk Cho; Korean J Pathol. 1992;26(1):40-52.

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Abstract PDF: Papillary and solid epithelial neoplasm of the pancreas from five patients were studied using immunohistochemistry and electron microscopy to define the cellular origin of this type of tumor. The tumors ranged in diameter form 5.5 to 15 cm Grossly, these were well circumscribed by a firm, gray-white, fibrous capsule and their cut-surface showed mainly area containing mucinous substance with necrotic and hemorrhagic material, with some solid portion. Microscopically, there was a solid and papillary pattern, with uniform cells typically having round to ovoid nuclei containing indistinct nucleoli and eosinophilic, granular cytoplasm. Within the cytoplasm of the tumor cells, numerous PAS-positive granules were found. Immunostaining was positive for neuron-specific enolase(three of five cases), alpha1-antitrypsin and alpha1-antichymotrypsin(three of five cases) in the solid and papillary portion of the tumor. But no polypeptide hormone immunoreactive cells were present in all cases except for gastrin which showed focally weak positivity in the papillary area. Ultrastructurally, the papillary and solid epithelial neopasm of the pancreas showed evidence of acinar cell differentiation, because in the cell of one observed some zymogen-like granules and presence of annulate lamellae. But also, abundant typical neurosecretory granules were detected in the tumor cells ultrastructurally. Both facts suggested acinar and islet cell differentiation of the tumor. From the these findings, it concluded that papillary and solid epithelial neoplasm of the pancreas may be originated from a primordial cell which will be able to render both endocrine and exocine component.

Case Report

Agenesis of the Dorsal Pancreas: An autopsy case.: Won Sang Park, Ki Hwa Yang, Seok Jin Kang, Byoung Kee Kim, Sun Moo Kim; Korean J Pathol. 1992;26(1):71-75.

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Abstract PDF: Agenesis of the dorsal pancreas is one of the rare congenital anomalies of the pancreas. Six cases of them have been reported. We have experienced an autopsy case of agenesis of the dorsal pancreas associated with fetal death in the uterus. Grossly, the body and tail of the pancreas and uncinate process were not found and those were partially replaced by adipose tissue. No abnormality was noted in the other organs. Microscopically, pancreatic tissue with autolytic change was identified only in the head portion of the pancreas.

Original Article

Serous Cystadenoma of the Pancreas.: Young Mee Cho, Gyung Yub Gong, Ghee Young Choe, Eun Sil Yu, In Chul Lee; Korean J Pathol. 1994;28(5):522-527.

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Abstract PDF: Pathologists and others have been calling the serous cystadenoma of the pancreas "microcystic cystadenoma", following Compagno and Oertel's proposal in l978 because it usually consists of innumerable small cysts (less than 2cm). However, unilocular or predominantly macrocystic types of serous cystadenoma have been occasionally reported. Therefore, they present a difficulty in precise preoperative and intraoperative diagnosis for their simi1ar gross appearance to other cystic lesions of the pancreas. We discovered two cases of macrocystic and unilocular serous cystadenomas of the pancreas which were lined by cuboidal to flattened epithelial cells. They contained PAS positive and D-PAS negative intracytoplasmic glycogen granules. The unilocular, macrocystic and microcystic patterns may represent a morphologic spectrum of a pancreatic neoplasm. So we propose to use the term "serous cystadenoma" rather than microcystic cystadenoma.

Case Report

Sarcomatoid Carcinoma of the Pancreas: A Case Report.: Kyung Hee Kim, Dae Young Kang, Min Koo Lee, Hyeon Woong Yang, Hyun Young Han; Korean J Pathol. 2006;40(4):306-310.

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Abstract PDF: We report here on an unusual case of a 73-year-old Korean female with sarcomatoid carcinoma. This tumor was composed of pancreatic ductal adenocarcinoma and a malignant fibrous histiocytoma-like stroma. The CT imaging revealed a multiseptated heterogenous hypodense mass that was 15 cm in size. The mass was located in the body and tail of the pancreas, spleen and gastrosplenic area. The pathologic examination showed that the carcinomatous component was negative for vimentin, and the sarcomatous component was positive for vimentin and CD 68. The ultrastructural examination showed that both the carcinomatous and sarcomatous components had desmosomes at the cell-cell contact sites. The patient refused postoperative adjuvant chemotherapy and she died of cachexia with generalized tumor extension about 3 months later. This report presents special data that can clarify the clinicopathological features and pathogenesis of this rare neoplasm.

Original Article

Prevalence and Clinicopathologic Features of Mucinous Cystic Tumor and Intraductal Papillary Mucinous Tumor of Pancreas in Korea.: Jin Hee Sohn, Kyung Me Kim, Seoung Wan Chae, Woo Ho Kim, Woo Sung Moon, Young Nyun Park, Chul Gun Park, Eun sil Yu, Hee Kyung Jang, Hee Jin Jang, Jong Jae Jung, Jin Sook Jung, So Young Jin, Jong Sang Choi, Dae Young Kang; Korean J Pathol. 2003;37(4):270-278.

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Abstract PDF: BACKGROUND
Mucin producing cystic neoplasms, such as mucinous cystic tumor (MCT) and intraductal papillary mucinous tumor (IPMT) of the pancreas, are uncommon but become increasing in their incidences. The pathologic classification and biologic potential of these neoplasmsremain the subject of controversy.
METHODS
The Gastrointestinal Pathology Study Group of the Korean Society of Pathologists analyzed the clinicopathologic characteristics of 85 casesof MCT and 72 cases of IPMT and examined the expression patterns of p53, CEA and MUC1.
RESULTS
IPMT was located largely in the head, and showed connection with the main pancreatic duct (MPD, 68.1%), no ovarian-like stroma (0/72), and presence of intervening intratumoralnormal or atrophic parenchyma. On the other hand, MCT was located largely in thetail (73%), and showed common ovarian-like stroma (66/80), rare connection with the MPD(7/85) and no intervening pancreatic parenchyma. CEA and p53 immunoexpressions weresignificantly increased from adenoma through borderline to carcinoma, but MUC 1 was expressedonly in the invasive carcinoma among cases of MCT and IPMT.
CONCLUSIONS
The tumorlocation, ovarian-like stroma, connection with the MPD and intratumoral intervening nonneoplastictissue were helpful in the differential diagnosis between IPMT and MCT. CEA and p53expressions can be indicators of malignancy, while MUC 1 expression can indicate invasion.

Case Report

Retroperitoneal Duplication Cyst Associated with Heterotopic Pancreas: A case report.: So Yeong Oh, Myoung Ja Chung, Dong Geun Lee, Ho Yeul Choi; Korean J Pathol. 1998;32(9):687-690.

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Abstract: Occurrence of a retroperitoneal duplication cyst associated with a heterotopic pancreas is rare. We report a case of a retroperitoneal duplication cyst associated with a heterotopic pancreas. A 35-year-old male was admitted, presenting with back pain. A pelvic computed tomographic scan disclosed a 10 cm-sized cystic mass filling the lower pelvis and displacing the rectosigmoid colon anteriorly. Histologically, the cyst wall was lined partly by mucin-secreting columnar epithelium, showing atypical hyperplasia and partly by a gastric fundic-type and a colonic-like mucosa. Beneath the epithelium, organized bundles of smooth muscle were arranged in two layers analogous to smooth muscles layers of the bowel and a small piece of pancreatic tissue were present in the smooth muscle wall of the cyst.

Original Article

Clinicopathological Analysis of Growth Patterns of Malignant Intraductal Papillary Mucinous Tumors of the Pancreas: Unusual Growth Pattern of Fistulous Extension.: Kee Taek Jang, Ghee Young Kwon, Geunghwan Ahn; Korean J Pathol. 2007;41(1):38-43.

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Abstract PDF: BACKGROUND
Usually, a malignant intraductal papillary mucinous tumor (IPMT) of the pancreas shows invasive carcinoma. Recently, IPMT with an unusual growth pattern of a fistulous extension was reported. However, little is known about malignant IPMTs with a different growth pattern of invasion and fistulous extension.
METHODS
Malignant IPMTs were classified into invasive (colloid or tubular type) carcinomas and the fistulous extension type according to their growth patterns. Their clinicopathological characteristics were compared.
RESULTS
Among a total of 68 cases of IPMT, there were 16 cases with malignant IPMT; eight, six and two of the colloid, tubular, and fistulous extension types, respectively. The immunohistochemical (IHC) expression of MUC1 was found in seven out of eight colloid and five out of six tubular types, but there was no IHC expression of MUC1 in the fistulous extension type. The IHC expression of MUC2 was noted in one of the eight colloid, one of the six tubular and in both cases with the fistulous extension type. There was no difference in the tumor recurrence rates bet- ween the different growth patterns.
CONCLUSIONS
IPMT with the fistulous extension type has a peculiar extension pattern consisting of multiple fistulous tracts without a mass. Although most of the epithelium in the fistulous tract show moderate to severe dysplasia, only the fistulous extension should be considered to be an unusual growth pattern of malignant IPMT. The clinical significance of this unusual type of IPMT remains to be determined.

Case Report

A Pancreatic Cyst of the Anterior Mediastinum.: Kye Weon Kwon, Kyung Young Jung, Woo Ick Yang; Korean J Pathol. 1998;32(11):1025-1027.

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Abstract: Although heterotopia of pancreatic tissue is a developmental anomaly found in approximately 2% of all autopsies, pancreatic tissue within the thorax and mediastinum is uncommon. In most of these instances, the pancreatic tissue is a component of gastroenteric duplication cysts, intralobar pulmonary sequestrations or teratomas. We describe an anterior mediastinal cyst consisting entirely of pancreatic tissue. A previously healthy 27-year-old woman was admitted due to chest pain during deep inspiration. The computed tomographic scan of the thorax showed a large cyst occupying the right anterior mediastinum. The excised multilocular cystic lesion measured 12 cm in maximum diameter and contained a brown, turbid fluid. The wall was fibrotic and showed a haphazard mixture of ducts and exocrine acini without islets. The histogenesis of this lesion is unclear.

Original Articles

Development of Endocrine Cells of Human Pancreases: A Morphologic and Immunohistochemical Study.: Jung Hee Cho, Je Geun Chi; Korean J Pathol. 1989;23(1):43-50.

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Abstract PDF: Pancreases from 123 human embryos and fetuses ranging from 3 weeks to 40 weeks of gestation age were studied. Thirty four cases were examined by immunohistochemical preparations to investigate the temporal development of the pancreatic islet particularly with reference of its origin, time of appearance and interrelation of various types of islet cells. Following results were made. 1) The endocrine cells and the acinar cells both originated from the primitive pancreatic duct cells. 2) Alpha, beta and delta cells began to appear almost simultaneously at 10 weeks og gestation. A, B and D cells, in decreasing order of frequency, were all found in the entire areas of the pancreas. 3) Four types of fetal islets i.e., preimtive, "homologous", bipolar and mantle, could be recognized during development. The latter 3 types first appeared simultaneously at 17 weeks of gestation. They continued to increase in sized and number throughout the fetal life and constituted permanent islets. 4) Two types of islet cells could be distinguished with routine H&E staining after the second trimester. 5) The size of fetal pancreatic islets were rapidly increased between early (64 micrometer) and mid-term (113 micrometer) (p<0.05). 6) The primitive islets were composed of 37.3% of alpha cells, 41.3% of beta cells and 13.4% of delta cells. At mid-term the mantle islets were composed of 36.4% of alpha cell, 42.7% of beta cell and 25.6% of delta cell. At term beta cell was higher value (52.6%) than alpha cell (29.3%) and delta cell (17.6%) (p<0.05).

Fine Needle Aspiration Cytology of Osteoclastic Giant Cell Tumor of the Pancreas.: Sun Hee Sung, Woon Sup Han; J Pathol Transl Med. 1998;9(1):89-94.

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Abstract PDF: A case of fine needle aspiration cytology of an osteoclastic giant cell tumor of pancreas, which is an uncommon variant of ductal adenocarcinoma, is described. Aspirated tumor cells were characterized by three populations: (1) bland osteoclast like giant cells with multiple small, round nuclei with distinct nucleoli, and abundant cytoplasm, (2) individually scattered or loosely clustered medium sized mononuclear tumor cells, having fine chromatin, smooth nuclear membrane, often prominent nucleoli, and high N/C ratio, (3) bland or atypical spindle shaped cells. Osteoid like lacy material was also seen on cell block section. The immunohistochemical studies using paraffin embedded cell block section showed positivities for vimentin and lysozyme in both giant and mononuclear tumor cells. However, they were negative for cytokeratin, epithelial membrane antigen, S-100 protein, carcinoembryonic antigen, and p53.

Nesidioblastosis of the Pancreas.: Young Bae Kim, Jin Sook Jeong, Ahn Hong Choi; Korean J Pathol. 1992;26(5):484-489.

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Abstract PDF: The morphologic abnormalities of the endocrine pancreas that underlie persistent neonatal hyperinsulinemic hypoglycemia and are included under the heading "nesidioblastosis" appears to be heterogeneous. This characteristic morphologic finding is ductuloinsular complexes showing endocrine cells budding off the ductoepithelium and merging with adjacent endocrine cell clusters. A case of nesidioblastosis associated with hyperinsulinemic hypoglycemia occurred in a 6/365 year-old male neonate. Microscopic finding of near totally resected pancreas revealed irregular sized islets and ductuloinsular complexes, both of which contained hypertrophied B cells with a few mitosis. Because of persistent hypoglycemia after first operation, he received second operation 8 days after. This histologic finding was more severe comparative to that of first operation. According to these findings, the pathogenesis of nesidioblastosis may be congenital or developmental defect of a kind of compensatory mechanism by unknown stimuli to acquire persistent hypoglycemia.

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