Journal of Pathology and Translational Medicine

Case Studies

A rare goblet cell adenocarcinoma arising from Barrett’s esophagus: the first reported case in the esophagus: Chi Eun Oh, Sung Eun Kim, Sun-Ju Oh; J Pathol Transl Med. 2024;58(2):81-86. Published online January 8, 2024; DOI: https://doi.org/10.4132/jptm.2023.12.26

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Abstract PDF: Goblet cell adenocarcinoma (GCA) is a rare and distinctive amphicrine tumor comprised of goblet-like mucinous cells and neuroendocrine cells. It is believed to originate from pluripotent stem cells located at the base of crypts. GCA predominantly arises from the appendix, with a few reported cases in extra-appendiceal locations such as the colorectum, small intestine, and stomach. In this case report, we present a unique instance of a 64-year-old male who initially received a diagnosis of neuroendocrine carcinoma in the distal esophagus based on biopsy but, following resection, was subsequently re-diagnosed with GCA arising from Barrett’s esophagus.

Primary testicular carcinoid tumor with marked lymphovascular invasion: Hyun Jung Lee, Joon Young Park, So Young Kim, Chung Su Hwang, Jung Hee Lee, Dong Hoon Shin, Jee Yeon Kim; J Pathol Transl Med. 2021;55(6):410-414. Published online October 20, 2021; DOI: https://doi.org/10.4132/jptm.2021.09.11

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Testicular carcinoid tumors are very rare, accounting for less than 1% of all testicular tumors. We report a rare case of a testicular carcinoid tumor with extensive lymphatic invasion. A 42-year-old man presented with a painless, enlarged right testicular mass. There was no history of injury or discomfort in this region. Right radical orchiectomy was performed, which showed a well-defined, non-encapsulated solid white mass with calcification (7.0 × 4.5 × 3.5 cm) and absence of cystic components. Microscopic examination using hematoxylin and eosin staining of the tumor sections identified organoid, trabecular, and solid patterns with rosette formation. Extensive multifocal lymphatic invasion was observed. Immunohistochemistry was positive for synaptophysin, chromogranin, and CD56. Testicular carcinoid tumors usually show good prognoses; however, there was extensive lymphovascular invasion in this case. Thus, in the case of unusual presentation of the disease, close follow-up is necessary.

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Testicular Primary Well-Differentiated Neuroendocrine Tumor: Clinicopathologic, Immunohistochemical, and Molecular Characterization of Two Patients
Liwei Jia, Bo Zhang, Daniel Shen, Prasad R. Koduru
International Journal of Surgical Pathology.2024; 32(8): 1574. CrossRef

Primary carcinoid tumor in the external auditory canal: Dong Hae Chung, Gyu Cheol Han, Na Rae Kim; J Pathol Transl Med. 2020;54(2):184-187. Published online November 13, 2019; DOI: https://doi.org/10.4132/jptm.2019.11.07

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Abstract PDF

A 39-year-old man visited the department of otolaryngology due to an ongoing hearing disturbance that had lasted for 1 year. Temporal bone computed tomography revealed soft tissue density nearly obliterating the left external auditory canal (EAC). The mass was composed of sheets of round tumor cells containing moderate amounts of fine granular cytoplasm and salt and pepper chromatin. Neither mitosis nor necrosis was found. The Ki-67 proliferation index was less than 2%. Cells were positive for CD56 and synaptophysin but negative for chromogranin, cytokeratin (CK) 20, and CK7. Based on these findings, the tumor was diagnosed as a carcinoid tumor, well differentiated neuroendocrine carcinoma, grade 1 (G1) according to current World Health Organization (WHO) classification of head and neck tumors; and a neuroendocrine tumor, G1 according to neuroendocrine neoplasm (NEN)-2018 WHO standard classification. He remained free of local recurrence and metastasis after 20 months of follow up. To date, only six cases of primary NENs in the EAC have been reported. Metastatic tumor should be included in the differential diagnoses. Because of its rarity, the prognosis and treatment have not yet been clarified.

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Incidental finding of a neuroendocrine neoplasm in a suspected ear canal exostosis
Alexander Wieck Fjaeldstad, Gerda Elisabeth Villadsen, Gitte Dam, Stephen Jacques Hamilton-Dutoit, Thomas Winther Frederiksen
Otolaryngology Case Reports.2022; 22: 100394. CrossRef
68Ga-DOTATATE Uptake in Well-Differentiated Neuroendocrine Tumor of the External Auditory Canal
Özge Erol Fenercioğlu, Ediz Beyhan, Rahime Şahin, Mehmet Can Baloğlu, Tevfik Fikret Çermik
Clinical Nuclear Medicine.2022; 47(8): e552. CrossRef

Original Articles

Morphologic Analysis of Pulmonary Neuroendocrine Tumors: Seung Seok Lee, Myunghee Kang, Seung Yeon Ha, Jungsuk An, Mee Sook Roh, Chang Won Ha, Jungho Han; Korean J Pathol. 2013;47(1):16-20. Published online February 25, 2013; DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.1.16

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Abstract PDF

Background

Few studies on how to diagnose pulmonary neuroendocrine tumors through morphometric analysis have been reported. In this study, we measured and analyzed the characteristic parameters of pulmonary neuroendocrine tumors using an image analyzer to aid in diagnosis.

Methods

Sixteen cases of typical carcinoid tumor, 5 cases of atypical carcinoid tumor, 15 cases of small cell carcinoma, and 51 cases of large cell neuroendocrine carcinoma were analyzed. Using an image analyzer, we measured the nuclear area, perimeter, and the major and minor axes.

Results

The mean nuclear area was 0.318±0.101 µm² in typical carcinoid tumors, 0.326±0.119 µm² in atypical carcinoid tumors, 0.314±0.107 µm² in small cell carcinomas, and 0.446±0.145 µm² in large cell neuroendocrine carcinomas. The mean nuclear circumference was 2.268±0.600 µm in typical carcinoid tumors, 2.408±0.680 µm in atypical carcinoid tumors, 2.158±0.438 µm in small cell carcinomas, and 3.247±1.276 µm in large cell neuroendocrine carcinomas. All parameters were useful in distinguishing large cell neuroendocrine carcinoma from other tumors (p=0.001) and in particular, nuclear circumference was the most effective (p=0.001).

Conclusions

Pulmonary neuroendocrine tumors showed nuclear morphology differences by subtype. Therefore, evaluation of quantitative nuclear parameters improves the accuracy and reliability of diagnosis.

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Clinical Significance of Persistent Tumor in Bone Marrow during Treatment of High-risk Neuroblastoma
Young Bae Choi, Go Eun Bae, Na Hee Lee, Jung-Sun Kim, Soo Hyun Lee, Keon Hee Yoo, Ki Woong Sung, Hong Hoe Koo
Journal of Korean Medical Science.2015; 30(8): 1062. CrossRef
Morphologic Alteration of Metastatic Neuroblastic Tumor in Bone Marrow after Chemotherapy
Go Eun Bae, Yeon-Lim Suh, Ki Woong Sung, Jung-Sun Kim
Korean Journal of Pathology.2013; 47(5): 433. CrossRef

Clinicopathological Analysis of 21 Thymic Neuroendocrine Tumors: Soomin Ahn, Jae Jun Lee, Sang Yun Ha, Chang Ohk Sung, Jhingook Kim, Joungho Han; Korean J Pathol. 2012;46(3):221-225. Published online June 22, 2012; DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.3.221

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Abstract PDF

Background

Thymic neuroendocrine carcinomas (NECs) are uncommon, for which there is no established information available because of a limited number of epidemiological study in Asia.

Methods

We reviewed 21 cases of surgically resected thymic NECs, and evaluated their pathological and clinical features.

Results

It showed male predominance (male/female ratio, 15/6) with wide age range from 20 to 72 years (mean age, 49 years). All 21 cases were divided into two types according to the World Health Organization criteria: atypical carcinoid (n=18) and large cell NEC (n=3). Three cases of atypical carcinoid (AC) were associated with ectopic Cushing's syndrome. All the patients (3/3) with large cell NEC (3/3) and 16.7% (3/18) of those with AC died of tumor progression. Common sites of metastasis included lung, lymph node, brain, lumbar spine, mediastinum, bone, and liver.

Conclusions

In conclusion, thymic neuroendocrine tumors carry a poor prognosis. Regarding the tumor classification, our results showed that a vast majority of carcinoids in the thymus correspond to ACs. In addition, our results also indicate that typical carcinoid is a very rare entity. Some cases of AC exhibited a large size, solid pattern and they showed aggressive clinical behavior, which highlights the spectrum of histologic appearances of thymic NECs.

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Kumud Gupta, Ravindra K. Dewan, Vinitha Viswambharan Nair, Rajat Saxena, Shaleen Prasad
The Indian Journal of Chest Diseases and Allied Sciences.2022; 56(4): 255. CrossRef
A resected case of large cell neuroendocrine carcinoma of the thymus
Masashi Umeda, Takahiko Misao, Tomoya Senoh, Yoshinobu Shikatani, Motoi Aoe
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Treatment strategy and prognostic analysis of spinal metastases from thymomas: A retrospective study from a single center
Shuzhong Liu, Xi Zhou, An Song, Zhen Huo, Siyuan Yao, Yipeng Wang, Yong Liu
Clinical Neurology and Neurosurgery.2020; 196: 106056. CrossRef
Large Cell Neuroendocrine Carcinoma of the Mediastinum Successfully Treated with Systemic Chemotherapy after Palliative Radiotherapy
Takeaki Hidaka, Saki Okuzumi, Ako Matsuhashi, Hidenori Takahashi, Kazunori Hata, Seiichiro Shimizu, Yoshinobu Iwasaki
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Surgical management of spinal metastases of thymic carcinoma
Shuzhong Liu, Xi Zhou, An Song, Zhen Huo, William A. Li, Radhika Rastogi, Yipeng Wang, Yong Liu
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Resected thymic large cell neuroendocrine carcinoma: A case report and review of the literature
Shogo Ogata, Ryo Maeda, Masaki Tomita, Yuichiro Sato, Takanori Ayabe, Kunihide Nakamura
International Journal of Surgery Case Reports.2019; 60: 53. CrossRef
Metastatic or locally advanced mediastinal neuroendocrine tumours
Aadil Adnan, Shreyas Kudachi, Sudha Ramesh, Kumar Prabhash, Sandip Basu
Nuclear Medicine Communications.2019; 40(9): 947. CrossRef
Results of treatment for thymic neuroendocrine tumours: multicentre clinicopathological study†
Naoko Ose, Hajime Maeda, Masayoshi Inoue, Eiichi Morii, Yasushi Shintani, Hiroshi Matsui, Hirohito Tada, Tositeru Tokunaga, Kenji Kimura, Yasushi Sakamaki, Yukiyasu Takeuchi, Kenjiro Fukuhara, Hiroshi Katsura, Teruo Iwasaki, Meinoshin Okumura
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Patterns of Failure Following Postoperative Radiation Therapy Based on “Tumor Bed With Margin” for Stage II to IV Type C Thymic Epithelial Tumor
Kyung Hwa Lee, Jae Myoung Noh, Yong Chan Ahn, Dongryul Oh, Jhingook Kim, Young Mog Shim, Jung-ho Han
International Journal of Radiation Oncology*Biology*Physics.2018; 102(5): 1505. CrossRef
Resected thymic large cell neuroendocrine carcinoma: report of a case
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Incidental metastatic mediastinal atypical carcinoid in a patient with parathyroid adenoma: a case report
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Alessandra Fabbri, Mara Cossa, Angelica Sonzogni, Paolo Bidoli, Stefania Canova, Diego Cortinovis, Maria Ida Abbate, Fiorella Calabrese, Nazarena Nannini, Francesca Lunardi, Giulio Rossi, Stefano La Rosa, Carlo Capella, Elena Tamborini, Federica Perrone,
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Retrosternal goiter and thymic carcinoid: A rare co-existence
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A case of large-cell neuroendocrine carcinoma of the thymus involving a patient with long-term survival after surgery
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Outcome of primary neuroendocrine tumors of the thymus: A joint analysis of the International Thymic Malignancy Interest Group and the European Society of Thoracic Surgeons databases
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Clinical Significance of Persistent Tumor in Bone Marrow during Treatment of High-risk Neuroblastoma
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Journal of Korean Medical Science.2015; 30(8): 1062. CrossRef
Tumor genetics and survival of thymic neuroendocrine neoplasms: A multi‐institutional clinicopathologic study
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Disseminated large cell neuroendocrine carcinoma associated with ectopic adrenocorticotropic hormone secretion
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The Interobserver Variability for Diagnosing Pulmonary Carcinoid Tumor.: Chang Hun Lee, Hee Kyung Chang, Hyoun Wook Lee, Dong Hoon Shin, Mee Sook Roh; Korean J Pathol. 2010;44(3):267-271.; DOI: https://doi.org/10.4132/KoreanJPathol.2010.44.3.267

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Abstract PDF

BACKGROUND
Although the grade of pulmonary carcinoid tumor is routinely reported in pathology practice, there is a paucity of data on the level of agreement between pathologists.
METHODS
Data for 30 cases of surgically resected pulmonary tumors diagnosed as carcinoid tumors (19 typical carcinoids [TCs] and 11 atypical carcinoids [ACs]) were retrieved from four university hospitals. These cases were independently evaluated by five pathologists and were classified according to the 2004 World Health Organization (WHO) classification. Agreement was regarded as "unanimous" if all five pathologists agreed, and as a "majority" if four agreed. The kappa statistic was calculated to measure the degree of agreement between pathologists.
RESULTS
Unanimous agreement was achieved for 50.0% and a majority agreement for 83.3% of the 30 cases. The range of the kappa values extended from 0.37 to 0.89. After a consensus meeting, there was disagreement between the original diagnosis by each institute and the consensus diagnosis by the five pathologists for 40.0% of the 30 cases. Based on the consensus diagnosis, the agreement was greater for TCs than that for ACs.
CONCLUSIONS
Discriminating carcinoid tumors is subject to interobserver variability. This study indicates that there is a need for more careful standardization and application of diagnostic criteria for making the diagnosis of pulmonary carcinoid tumor.

Citations

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Assessment of the current and emerging criteria for the histopathological classification of lung neuroendocrine tumours in the lungNENomics project
É. Mathian, Y. Drouet, A. Sexton-Oates, M.G. Papotti, G. Pelosi, J.-M. Vignaud, L. Brcic, A. Mansuet-Lupo, F. Damiola, C. Altun, J.-P. Berthet, C.B. Fournier, O.T. Brustugun, G. Centonze, L. Chalabreysse, V.T. de Montpréville, C.M. di Micco, E. Fadel, N.
ESMO Open.2024; 9(6): 103591. CrossRef
Limited additive value of the Ki‐67 proliferative index on patient survival in World Health Organization‐classified pulmonary carcinoids
Dorian R A Swarts, Martina Rudelius, Sandra M H Claessen, Jack P Cleutjens, Stefan Seidl, Marco Volante, Frans C S Ramaekers, Ernst J M Speel
Histopathology.2017; 70(3): 412. CrossRef
Interobserver Variability for the WHO Classification of Pulmonary Carcinoids
Dorian R.A. Swarts, Robert-Jan van Suylen, Michael A. den Bakker, Matthijs F.M. van Oosterhout, Frederik B.J.M. Thunnissen, Marco Volante, Anne-Marie C. Dingemans, Marc R.M. Scheltinga, Gerben P. Bootsma, Harry M.M. Pouwels, Ben E.E.M. van den Borne, Fran
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Lung parenchymal invasion in pulmonary carcinoid tumor: An important histologic feature suggesting the diagnosis of atypical carcinoid and poor prognosis
Sang Yun Ha, Jae Jun Lee, Junhun Cho, Jiyeon Hyeon, Joungho Han, Hong Kwan Kim
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CD44 and OTP Are Strong Prognostic Markers for Pulmonary Carcinoids
Dorian R.A. Swarts, Mieke E.R. Henfling, Leander Van Neste, Robert-Jan van Suylen, Anne-Marie C. Dingemans, Winand N.M. Dinjens, Annick Haesevoets, Martina Rudelius, Erik Thunnissen, Marco Volante, Wim Van Criekinge, Manon van Engeland, Frans C.S. Ramaeke
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Altered expression of microRNA miR‐21, miR‐155, and let‐7a and their roles in pulmonary neuroendocrine tumors
Hyoun Wook Lee, Eun Hee Lee, Seung Yeon Ha, Chang Hun Lee, Hee Kyung Chang, Sunhee Chang, Kun Young Kwon, Il Seon Hwang, Mee Sook Roh, Jeong Wook Seo
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Differential expression of forkhead box M1 and its downstream cyclin‐dependent kinase inhibitors p27kip1 and p21waf1/cip1 in the diagnosis of pulmonary neuroendocrine tumours
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Case Report

Primary Atypical Carcinoid Tumor of Liver: A case report.: Won Ae Lee, Hong Yong Kim, Ill Hyang Ko; Korean J Pathol. 1995;29(6):807-810.

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Abstract PDF: Primary hepatic carcinoid tumors are extremely rare although the liver is a frequent site of metastases from intestinal carcinoids. Recently we investigated a case of primary hepatic atypical carcinoid in a 47-year-old man who had infested with Clonorchis sinensis for 20 years. The resected right lobe of the liver was almost completely occupied by a huge tumor, measuring 20 x 19 x 12 cm. The cut surfaces of the mass were solid, soft and pale yellow, accompanied by several small satellite nodules, measuring up to 1.5 cm in diameter. Microscopically, the tumor consisted of polygonal to columnar cells with eosinophilic granular cytoplasm forming Lym-numerous small acini and large trabeculae. Their nuclei were round to polygonal with coarse stone chromatin, had obscure to small nucleoli and frequent mitoses. There were multiple necrotic foci of varing sizes. The surrounding dilated bile ducts contained several degenerating worms on in of Clonorchis sinensis. The tumor cells were argyrophil-positive but argentaffin-negative. Immunohistochemically, the tumor cells were positive for cytokeratin, chromogranin and somatostatin but were negative for CEA, AFP, insulin, glucagon, ACTH, growth hormone and volve-prolactin. Ultrastructually, the tumor cells contained variable-sized numerous electron dense of neurosecretory granules.

Original Article

Carcinoid Tumors of the Gastrointestinal Tract: Analysis of 36 Cases.: Kyeong Cheon Jung, Hee Sung Kim, Sang Yong Song, Ghee Young Choe, Yong Il Kim; Korean J Pathol. 1996;30(5):396-407.

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Abstract PDF: We reviewed a total of 36 cases of gastrointestinal carcinoid tumors during a period of ten and a half years at Seoul National University Hospital, looking at their histologic and ultrastructural features, as well as immunohistochemical results, then we correlated these with location. The primary sites of carcinoid tumors were rectum(39%), stomach(25%), appendix(22%), small intestine(8%) and colon(6%), in order of frequency. The predominant histologic types by organ were mixed and solid(A) types in all location, the solid type(type A) in the stomach, and tubular type(type C) in the rectum. Six out of 8 cases of appendiceal carcinoid were type A and the other two cases were mucinous carcinoid. Tubular adenoma or adenocarcinoma was associated with 36% of the rectal carcinoids, with one case of carcinoid in the small intestine, and one(11%) of the gastric carcinoids was found near an adenocarcinoma. Metastasis was found in one case of ileal carcinoid(100%), one case of cecal carcinoid, 3 of gastric carcinoid(33%) and 2 of rectal carcinoid(14%). Primary tumor size ranged from 1 to 7 cm. Tumor necrosis and endolymphatic tumor emboli were found in 57% and 43% of the metastatic carcinoids, respectively. PCNA index showed a borderline significant difference between metastatic and nonmetastatic groups(P value=0.063). The low prevalence of appendiceal carcinoid and the relatively high prevalence of gastric-rectal carcinoid may reflect a low chance of incidental appendectomy and frequent detection of gastrointestinal endoscopy.

Case Reports

Goblet Cell Carcinoid of the Appendix: A case report.: Joo Heon Kim, Ho Lee, So Young Oh, Myoung Jae Kang, Ho Yeul Choi, Dong Geun Lee; Korean J Pathol. 1996;30(9):839-842.

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Abstract PDF: Carcinoid tumors of the appendix are common incidental findings, but appendiceal tumors with histologic features of both carcinoids and adenocarcinomas are rare, and their biologic behavior and histogenesis are still unclear. We report a case of goblet cell carcinoid of the appendix in a 54-year-old male, who exhibited pain in the right lower abdomen. Microscopically, the tumor contained smooth-bordered, widely separated nests composed of tumor cells with abundant mucin. The principal tumor cell type had a close resemblance to the normal goblet cell. Histochemically, the tumor cells revealed positive reaction for PAS and alcian blue stain. Immunohistochemically, the tumor showed strong reactivity for carcinoembryonic antigen, chromogranin and, neuron specific enolase but none for cytokeratin and epithelial membrane antigen.

Carcinoid Tumor Arising in a Horseshoe Kidney: A case report.: Yoo Jin Kim, Bang Hur, Man Ha Hur; Korean J Pathol. 1996;30(12):1129-1137.

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Abstract PDF: Primary carcinoid tumor of the kidney is extremely rare ; only 29 previous reports have been described in the world literature, 3 cases of which were known to arise in horseshoe kidneys. Because of the scarcity of primary renal carcinoid tumor, its clinicopathologic features and prognosis are not well characterized, and its histogenesis is unknown. We present a case of primary carcinoid tumor arising in the horseshoe kidney of a 43-year-old man. The lesion is morphologically identical to those of gastrointestinal or respiratory tracts. In addition, this tumor contains clusters of intestinal epithelium with goblet cells and mature bony trabeculae. The cytoplasm of the tumor cells are immunoreactive for keratin, NSE, and synaptophysin, but are negative with Grimelius and Fontana-Masson stains. Membrane-bound cytoplasmic neurosecretory granules are observed by electron microscopic examination. This is the first case of primary renal carcinoid tumor arising in a horseshoe kidney documented in Korea. We describe the gross features, light and electron microscopic, and immunohistochemical findings of renal carcinoid and review the literature, with speculations on the possible histogenesis of this unusual neoplasm.

Combined Mucinous Tumor and Carcinoid of Appendix Associated with Mucinous Tumor of Ovary and Pseudomyxoma Peritonei: A case report .: Hye Jeong Choi, Mi Jin Kim; Korean J Pathol. 1999;33(11):1094-1096.

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Abstract PDF: We report a case of mucinous cystadenoma of uncertain malignant potential and carcinoid of appendix associated with bilateral mucinous cystadenoma of ovary and pseudomyxoma peritonei. The patient was a 46-year-old female. She suffered from dyspnea and lower abdominal palpable masses for several months. Ultrasonogram showed multilocular huge ovarian cysts. Appendectomy, transabdominal hysterectomy, bilateral salphingo-oophorectomy and biopsy of omentum were performed. The bilateral ovaries measured 16 11 cm and 7X5 cm in size, respectively. The both ovaries showed multilocular cysts filled with thick mucus material. The ovarian cysts were covered by a single layer of columnar epithelium with focal proliferation. Mucus materials dissected through the ovarian stroma (pseudomyxoma ovarii). The tip of appendix was dilated and covered by mucus material. The cut surface showed a cyst and a yellowish solid mass proximal to the cyst. Microscopically, the appendiceal cyst was lined by stratified columnar epithelium with moderate cytologic atypia. Mucus material dissected through the wall. In the proximal portion of the appendix, a classic carcinoid with focal tubular form was present in submucosa and muscle layer. The omentum was covered by thick mucus material. Microscopically, the omentum showed mucinous epithelium and mucus material (pseudomyxoma peritonei).

Primary Carcinoid Tumor Arising in a Mature Teratoma of the Testis: A Case Report.: Jai Hyang Go; Korean J Pathol. 2001;35(1):86-88.

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Abstract PDF: Primary carcinoid tumor is rarely associated with mature teratoma in the testis. In the few cases reported, the primary carcinoid tumors in the testis were often microscopic in size. Although it has been generally accepted that carcinoids arising in teratomas have an indolent clinical course, some of these tumors have been reported to metastasize and cause death of the patient. Therefore, long term follow up may be warranted for patients having primary testicular carcinoid tumors irrespective of coexisting teratoma. We report a case of primary testicular carcinoid tumor of microscopic size incidentally found in mature cystic teratoma occurring in a 44 year-old male patient.

Calcitonin Producing Middle Ear Carcinoid with Amyloid Deposition: A Case Report.: Beom Jin Lim, Woo Ick Yang, Soon Won Hong, Chanil Park; Korean J Pathol. 2002;36(2):115-118.

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Abstract PDF: Carcinoid tumor of the middle ear cavity is a rare neoplasm of low malignant potential. It grows slowly and rarely metastasizes. We recently experienced a case of carcinoid tumor of the middle ear cavity occurring in a 51-year-old female who was presented with hearing loss and tinnitus of the right ear. A 1 cm sized pinkish soft mass was noted in the promontory of the middle ear. Histologically, the mass was composed of nests and cords of tumor cells with slightly pleomorphic nuclei and eosinophilic cytoplasm. The extracellular matrix was glassy pink, homogeneous and exhibited apple green birefringence under a polarized light microscope after Congo red staining. The tumor cells showed immunoreactivity for cytokeratin, chromogranin A, synaptophysin, neuron-specific enolase and calcitonin. Electron microscopic examination confirmed the presence of neurosecretory granules. The production of calcitonin and amyloid by the tumor cells suggests the possible relationship of this tumor to the thyroid C cells.

Fine Needle Aspiration Cytology of Atypical Carcinoid Tumor of the Lung: 2 Cases Report .: Youn Soo Lee, Gyeong Sin Park, Young Jin Choi, Seok Jin Kang, Byung Kee Kim, Sang In Shim; Korean J Cytopathol. 1997;8(1):76-82.

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Abstract PDF: Two cases of pulmonary atypical carcinoid tumor were diagnosed by fine needle aspiration cytology. Although the cytologic features of atypical carcinoid tumor have been relatively well described, it is easy to confuse atypical carcinoid tumor with typical carcinoid tumor, small cell carcinoma and adenocarcinoma of the lung. Atypical carcinoid tumor has been recognized as a distinct variant of pulmonary neuroendocrine carcinoma, with characteristic histopathologic and clinical features that separate it from both carcinoid and small cell carcinoma. The distinction of atypical carcinoid tumor from small cell carcinoma has important prognostic and therapeutic implications. The cytologic characteristics of atypical carcinoid tumor included polygonal to fusiform cells with a variable amount of lacy cytoplasm, oval nuclei with coarsely dispersed chromatin and frequent nucleoli, and mild pleomorphism. The malignant cells were arranged either in acinus-like clusters or in epithelial sheets.

Fine Needle Aspiration Cytology of a Thymic Carcinoid Tumor: A Case Report.: Young Ha Oh, Ki Seok Jang, Young Soo Song, Chul Burm Lee, Choong Ki Park, Moon Hyang Park, Yong Wook Park; Korean J Cytopathol. 2005;16(1):41-46.

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Abstract PDF: Carcinoid tumors of the thymus are vanishingly rare, and the characteristic cytologic findings of this condition have never before been reported in Korea. Recently, we encountered a 58-year-old woman who had been suffering from general weakness and weight loss for several months. Radiological imaging revealed a large anterior mediastinal mass. A fine needle aspiration biopsy (FNAB) of the mass showed predominantly scattered single cells, as well as some loose clusters of small cells with scanty cytoplasm. Some of these small cells exhibited plasmacytoid features, with moderately granular cytoplasm. We also discuss the cytological differential diagnosis between thymic carcinoid and other mediastinal tumors.

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