Journal of Pathology and Translational Medicine

Original Article

Adjunctive markers for classification and diagnosis of central nervous system tumors: results of a multi-center neuropathological survey in Korea: Yoon Jin Cha, Se Hoon Kim, Na Rae Kim; J Pathol Transl Med. 2020;54(2):165-170. Published online February 20, 2020; DOI: https://doi.org/10.4132/jptm.2020.02.04

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Background
The revised 4th 2016 World Health Organization (WHO) classification of tumors of the central nervous system (CNS) classification has adopted integrated diagnosis encompassing the histology and molecular features of CNS tumors. We aimed to investigate the immunohistochemistry, molecular testing, and testing methods for diagnosis of CNS tumors in pathological labs of tertiary centers in Korea, and evaluate the adequacy of tests for proper diagnosis in daily practice.
Methods
A survey, composed of eight questions concerning molecular testing for diagnosis of CNS tumors, was sent to 10 neuropathologists working in tertiary centers in Korea.
Results
For diagnosis of astrocytic and oligodendroglial tumors, all 10 centers performed isocitrate dehydrogenase mutations testing and 1p/19q loss of heterozygosity. For glioneuronal tumors, immunohistochemistry (IHC) assays for synaptophysin (n = 9), CD34 (n = 7), BRAF(VE1) (n = 5) were used. For embryonal tumors, particularly in medulloblastoma, four respondents used IHC panel (growth factor receptor bound protein 2-associated protein 1, filamin A, and yes-associated protein 1) for molecular subclassification. Regarding meningioma, all respondents performed Ki-67 IHC and five performed telomerase reverse transcriptase promoter mutation.
Conclusions
Most tertiary centers made proper diagnosis in line with 2016 WHO classification. As classification of CNS tumors has evolved to be more complex and more ancillary tests are required, these should be performed considering the effect of necessity and justification.

Citations

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Exploring the role of epidermal growth factor receptor variant III in meningeal tumors
Rashmi Rana, Vaishnavi Rathi, Kirti Chauhan, Kriti Jain, Satnam Singh Chhabra, Rajesh Acharya, Samir Kumar Kalra, Anshul Gupta, Sunila Jain, Nirmal Kumar Ganguly, Dharmendra Kumar Yadav, Timir Tripathi
PLOS ONE.2021; 16(9): e0255133. CrossRef

Case Study

Frozen Cytology of Meningeal Malignant Solitary Fibrous Tumor/Hemangiopericytoma: Myunghee Kang, Na Rae Kim, Dong Hae Chung, Gie-Taek Yie; J Pathol Transl Med. 2019;53(3):192-197. Published online April 11, 2019; DOI: https://doi.org/10.4132/jptm.2019.03.20

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Abstract PDF

A 51-year-old woman presented with severe dizziness. The brain magnetic resonance image revealed a 5.5 cm multiloculated mass with a thick rim in the left temporal lobe. Cytological examination of frozen diagnosis of the mass showed hypercellular sheets of round and rhabdoid cells in a hemorrhagic background, and two mitotic figures were observed. Histologically, the excised dura-based mass consisted of predominantly round cells with small foci of rhabdoid tumor cells in a pseudoalveolar pattern in a hemorrhagic background, and the cells showed nuclear positivity for signal transducer and activator of transcription 6 as well as frequent mitosis. The mass was diagnosed as a grade 3 solitary fibrous tumor (SFT)/hemangiopericytoma (HPC). The cytological diagnosis of SFT/HPC is challenging because of the heterogeneous cytological findings, such as histological heterogeneity, and because there are no standardized cytological criteria for malignant SFT/HPC. Cytological findings, such as singly scattered small cells, hypercellularity, rare ropy collagen, and round and rhabdoid cells with pseudoalveolar pattern, may assist in the diagnosis of malignant SFT/HPC.

Citations

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Cytologic features of mesenchymal, melanocytic and haematolymphoid tumours of the central nervous system and metastases
Carmen Bárcena, José A. Jiménez‐Heffernan
Cytopathology.2024; 35(5): 590. CrossRef
A Hemangiopericytoma in the External Auditory Canal: A Rare Clinical Presentation and Management
Vaibhavi Patil, Prasad Deshmukh, Sagar S Gaurkar , Ayushi Ghosh Moulic, Jasleen Kaur
Cureus.2024;[Epub] CrossRef
Scoring system for intraoperative diagnosis of intracranial schwannoma by squash cytology
Hirotaka Fujita, Takuma Tajiri, Tomohisa Machida, Nozomi Nomura, Suguru Toguchi, Hitoshi Itoh, Shinichiro Hiraiwa, Tomoko Sugiyama, Chie Inomoto, Masaaki Imai, Shinri Oda, Masami Shimoda, Naoya Nakamura
Cytopathology.2022; 33(2): 196. CrossRef
Occurrence of a solitary fibrous tumor adjacent to the resection bed of a high-grade meningioma: A case report
Coby Cunningham, Rocco Dabecco, Justin Davanzo
Interdisciplinary Neurosurgery.2021; 25: 101277. CrossRef
A case of solitary fibrous tumor arising in the meninge
Saori NAKANISHI, Naoto KURODA, Toshiko TAKAI, Mari KOJIMA, Misato OONOGI
The Journal of the Japanese Society of Clinical Cytology.2021; 60(4): 224. CrossRef
Intraoperative frozen cytology of intraosseous cystic meningioma in the sphenoid bone
Na Rae Kim, Gie-Taek Yie
Journal of Pathology and Translational Medicine.2020; 54(6): 508. CrossRef

Original Article

Intraoperative Frozen Cytology of Central Nervous System Neoplasms: An Ancillary Tool for Frozen Diagnosis: Myunghee Kang, Dong Hae Chung, Na Rae Kim, Hyun Yee Cho, Seung Yeon Ha, Sangho Lee, Jungsuk An, Jae Yeon Seok, Gie-Taek Yie, Chan Jong Yoo, Sang Gu Lee, Eun Young Kim, Woo Kyung Kim, Seong Son, Sun Jin Sym, Dong Bok Shin, Hee Young Hwang, Eung Yeop Kim, Kyu Chan Lee; J Pathol Transl Med. 2019;53(2):104-111. Published online January 14, 2019; DOI: https://doi.org/10.4132/jptm.2018.11.10

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Abstract PDF

Background
Pathologic diagnosis of central nervous system (CNS) neoplasms is made by comparing light microscopic, immunohistochemical, and molecular cytogenetic findings with clinicoradiologic observations. Intraoperative frozen cytology smears can improve the diagnostic accuracy for CNS neoplasms. Here, we evaluate the diagnostic value of cytology in frozen diagnoses of CNS neoplasms.
Methods
Cases were selected from patients undergoing both frozen cytology and frozen sections. Diagnostic accuracy was evaluated.
Results
Four hundred and fifty-four cases were included in this retrospective single-center review study covering a span of 10 years. Five discrepant cases (1.1%) were found after excluding 53 deferred cases (31 cases of tentative diagnosis, 22 cases of inadequate frozen sampling). A total of 346 cases of complete concordance and 50 cases of partial concordance were classified as not discordant cases in the present study. Diagnostic accuracy of intraoperative frozen diagnosis was 87.2%, and the accuracy was 98.8% after excluding deferred cases. Discrepancies between frozen and permanent diagnoses (n = 5, 1.1%) were found in cases of nonrepresentative sampling (n = 2) and misinterpretation (n = 3). High concordance was observed more frequently in meningeal tumors (97/98, 99%), metastatic brain tumors (51/52, 98.1%), pituitary adenomas (86/89, 96.6%), schwannomas (45/47, 95.8%), high-grade astrocytic tumors (47/58, 81%), low grade astrocytic tumors (10/13, 76.9%), non-neoplastic lesions (23/36, 63.9%), in decreasing frequency.
Conclusions
Using intraoperative cytology and frozen sections of CNS tumors is a highly accurate diagnostic ancillary method, providing subtyping of CNS neoplasms, especially in frequently encountered entities.

Citations

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Intraoperative Integrated Diagnostic System for Malignant Central Nervous System Tumors
Takahiro Hayashi, Kensuke Tateishi, Shinichiro Matsuyama, Hiromichi Iwashita, Yohei Miyake, Akito Oshima, Hirokuni Honma, Jo Sasame, Katsuhiro Takabayashi, Kyoka Sugino, Emi Hirata, Naoko Udaka, Yuko Matsushita, Ikuma Kato, Hiroaki Hayashi, Taishi Nakamur
Clinical Cancer Research.2024; 30(1): 116. CrossRef
A multicenter proof-of-concept study on deep learning-based intraoperative discrimination of primary central nervous system lymphoma
Xinke Zhang, Zihan Zhao, Ruixuan Wang, Haohua Chen, Xueyi Zheng, Lili Liu, Lilong Lan, Peng Li, Shuyang Wu, Qinghua Cao, Rongzhen Luo, Wanming Hu, Shanshan lyu, Zhengyu Zhang, Dan Xie, Yaping Ye, Yu Wang, Muyan Cai
Nature Communications.2024;[Epub] CrossRef
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Ali A. Mohamed, Emma Sargent, Cooper Williams, Zev Karve, Karthik Nair, Brandon Lucke-Wold
Tomography.2024; 10(5): 693. CrossRef
Unveiling the potential application of intraoperative brain smear for brain tumor diagnosis in low-middle-income countries: A comprehensive systematic review
Muhammad Shakir, Ahmed Altaf, Hawra Hussain, Syed Muhammad Aqeel Abidi, Zoey Petitt, Mahnoor Tariq, Ahmed Gilani, S. Ather Enam
Surgical Neurology International.2023; 14: 325. CrossRef
A Comparative Study of Squash Smear Cytology Diagnosis and Radiological Diagnosis with Histopathology in Central Nervous System Lesions
B N Kumarguru, G Santhipriya, S Kranthi Kumar, R Ramesh Kumar, A S Ramaswamy, P Janakiraman
Journal of Cytology.2022; 39(1): 1. CrossRef
Intraoperative squash cytology provides a qualitative intraoperative diagnosis for cases in which frozen section yields a diagnosis of equivocal brain tumour
Hirotaka Fujita, Takuma Tajiri, Tomohisa Machida, Nozomi Nomura, Suguru Toguchi, Hitoshi Itoh, Shinichiro Hiraiwa, Tomoko Sugiyama, Masaaki Imai, Shinri Oda, Masami Shimoda, Naoya Nakamura
Cytopathology.2020; 31(2): 106. CrossRef
Intraoperative frozen cytology of intraosseous cystic meningioma in the sphenoid bone
Na Rae Kim, Gie-Taek Yie
Journal of Pathology and Translational Medicine.2020; 54(6): 508. CrossRef
Use of 5-Aminolevulinic Acid for Confirmation of Lesional Biopsy Sample in Presumed High-Grade Glioma
Victoria L. Watson, Jeffrey W. Cozzens
World Neurosurgery.2019; 132: 21. CrossRef

Case Studies

Primary Peripheral Gamma Delta T-Cell Lymphoma of the Central Nervous System: Report of a Case Involving the Intramedullary Spinal Cord and Presenting with Myelopathy: Jeemin Yim, Seung Geun Song, Sehui Kim, Jae Won Choi, Kyu-Chong Lee, Jeong Mo Bae, Yoon Kyung Jeon; J Pathol Transl Med. 2019;53(1):57-61. Published online October 1, 2018; DOI: https://doi.org/10.4132/jptm.2018.08.21

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Abstract PDF

Primary central nervous system lymphoma of T-cell origin (T-PCNSL) is rare, and its clinicopathological features remain unclear. Peripheral T-cell lymphoma of γδ T-cell origin is an aggressive lymphoma mainly involving extranodal sites. Here, we report a case of γδ T-PCNSL involving the intramedullary spinal cord and presenting with paraplegia. A 75-year-old Korean woman visited the hospital complaining of back pain and lower extremity weakness. Magnetic resonance imaging revealed multifocal enhancing intramedullary nodular lesions in the thoracic and lumbar spinal cord. An enhancing nodular lesion was observed in the periventricular white matter of the lateral ventricle in the brain. There were no other abnormalities in systemic organs or skin. Laminectomy and tumor removal were performed. The tumor consisted of monomorphic, medium-to-large atypical lymphocytes with pale-to-eosinophilic cytoplasm. Immunohistochemically, the tumor cells were CD3(+), TCRβF1(-), TCRγ(+), CD30(-), CD4(-), CD8(-), CD56(+), TIA1(+), granzyme B(+), and CD103(+). Epstein-Barr virus in situ was negative. This case represents a unique T-PCNSL of γδ T-cell origin involving the spinal cord.

Citations

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B-Cell Lymphoma Intramedullary Tumor: Case Report and Systematic Review
Daniel Gregório Gonsalves, Paulo Eduardo Albuquerque Zito Raffa, Gabriela Gerenutti de Sousa, Melissa Esposito Gomes Rigueiral, Iracema Araújo Estevão, Cesar Cozar Pacheco, Roger Thomaz Rotta Medeiros, Paulo Roberto Franceschini, Paulo Henrique Pires de A
Asian Journal of Neurosurgery.2023; 18(02): 231. CrossRef
Primary intramedullary spinal cord lymphoma misdiagnosed as longitudinally extensive transverse myelitis: a case report and literature review
Huizhen Ge, Li Xu, Huajie Gao, Suqiong Ji
BMC Neurology.2023;[Epub] CrossRef
Clinicopathologic and Genetic Features of Primary T-cell Lymphomas of the Central Nervous System
Jeemin Yim, Jiwon Koh, Sehui Kim, Seung Geun Song, Jeong Mo Bae, Hongseok Yun, Ji-Youn Sung, Tae Min Kim, Sung-Hye Park, Yoon Kyung Jeon
American Journal of Surgical Pathology.2022; 46(4): 486. CrossRef
Peripheral T-Cell Lymphomas Involving the Central Nervous System: A Report From the Czech Lymphoma Study Group Registry
Heidi Mocikova, Robert Pytlík, Katerina Benesova, Andrea Janikova, Juraj Duras, Alice Sykorova, Katerina Steinerova, Vit Prochazka, Vit Campr, David Belada, Marek Trneny
Frontiers in Oncology.2022;[Epub] CrossRef

An Autopsy Case of Epstein-Barr Virus–Associated Diffuse Large B-Cell Lymphoma of the Central Nervous System in an Immunocompromised Host: Sun-Young Park, Seong Ik Kim, Hannah Kim, Yoojin Lee, Sung-Hye Park; J Pathol Transl Med. 2018;52(1):51-55. Published online August 4, 2017; DOI: https://doi.org/10.4132/jptm.2017.01.23

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Abstract PDF

Lymphomas arising in the central nervous system (CNS) of immunocompromised hosts are most commonly non-Hodgkin’s lymphomas and are highly associated with Epstein-Barr virus (EBV). Here we report an autopsy case of EBV-associated CNS diffuse large B-cell lymphoma (DLBCL) in a host suffering from systemic lupus erythematosus who underwent immunosuppressive therapy. After autopsy, EBV-associated CNS DLBCL as well as pulmonary mixed aspergillosis and Pneumocystis jirovecii pneumonia were added to the cause of clinical manifestations of complicated pneumonia and cerebral hemorrhage in this immunocompromised patient. In conclusion, complex disease processes were revealed by autopsy in this case, indicating that the clinicopathological correlations observed through autopsy can improve our understanding of disease progression and contribute to the management of similar patients in the future.

Citations

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Primary central nervous system lymphoma in neuropsychiatric systemic lupus erythematosus: case-based review
Takanori Ichikawa, Yasuhiro Shimojima, Dai Kishida, Tomoki Kaneko, Yoshiki Sekijima
Rheumatology International.2021; 41(5): 1009. CrossRef

Original Article

Meningeal Solitary Fibrous Tumors with Delayed Extracranial Metastasis: Nayoung Han, Hannah Kim, Soo Kee Min, Sun-Ha Paek, Chul-Kee Park, Seung-Hong Choi, U-Ri Chae, Sung-Hye Park; J Pathol Transl Med. 2016;50(2):113-121. Published online December 14, 2015; DOI: https://doi.org/10.4132/jptm.2015.10.30

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Abstract PDF

Background
The term solitary fibrous tumor (SFT) is preferred over meningeal hemangiopericytoma (HPC), because NAB2-STAT6 gene fusion has been observed in both intracranial and extracranial HPCs. HPCs are now considered cellular variants of SFTs. Methods: This study analyzes 19 patients with STAT6-confirmed SFTs, who were followed for over 11 years in a single institution. Ten patients (10/19, 56.2%) had extracranial metastases (metastatic group), while the remainder (9/19) did not (non-metastatic group). These two groups were compared clinicopathologically. Results: In the metastatic group, the primary metastatic sites were the lungs (n = 6), bone (n = 4), and liver (n = 3). There was a mean lag time of 14.2 years between the diagnosis of the initial meningeal tumor to that of systemic metastasis. The median age at initial tumor onset was 37.1 years in the metastatic group and 52.5 in the non-metastatic group. The 10-year survival rates of the metastatic- and non-metastatic groups were 100% and 33%, respectively. The significant prognostic factors for poor outcomes on univariate analysis included advanced age (≥45 years) and large initial tumor size (≥5 cm). In contrast, the patients with higher tumor grade, high mitotic rate (≥5/10 high-power fields), high Ki-67 index (≥5%), and the presence of necrosis or CD34 positivity showed tendency of poor prognosis but these parameters were not statistically significant poor prognostic markers. Conclusions: Among patients with SFTs, younger patients (<45 years) experienced longer survival times and paradoxically had more frequent extracranial metastases after long latent periods than did older patients. Therefore, young patients with SFTs require careful surveillance and follow-up for early detection of systemic metastases.

Citations

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Meningeal Solitary Fibrous Tumor: A Single-Center Retrospective Cohort Study
Siyer Roohani, Yasemin Alberti, Maximilian Mirwald, Felix Ehret, Carmen Stromberger, Soleiman Fabris Roohani, Katja Bender, Anne Flörcken, Sven Märdian, Daniel Zips, David Kaul, Manish Charan
Sarcoma.2024; 2024: 1. CrossRef
De-differentiation associated with drop metastasis of a recurrent intracranial solitary fibrous tumor: a case report and literature review
Chenhui Zhao, Xiran Fan, Wanwan Gao, Fan Zhang, Haijun Lv, Xiaochun Jiang, Guangfu Di
International Journal of Neuroscience.2022; 132(8): 843. CrossRef
Long-term extracranial metastatic relapse of an intraventricular solitary fibrous tumor: a case report
Tarek Assi, Elie Samaha, Hussein Nassereddine
Anti-Cancer Drugs.2022; 33(1): e764. CrossRef
Multidisciplinary Treatment of Liver Metastases from Intracranial SFTs/HPCs: A Report of Three Consecutive Cases
Felix J. Krendl, Franka Messner, Gregor Laimer, Angela Djanani, Andreas Seeber, Georg Oberhuber, Dietmar Öfner, Dominik Wolf, Stefan Schneeberger, Reto Bale, Christian Margreiter
Current Oncology.2022; 29(11): 8720. CrossRef
A review of solitary fibrous tumor/hemangiopericytoma tumor and a comparison of risk factors for recurrence, metastases, and death among patients with spinal and intracranial tumors.
Enrico Giordan, Elisabetta Marton, Alexandra M. Wennberg, Angela Guerriero, Giuseppe Canova
Neurosurgical Review.2021; 44(3): 1299. CrossRef
Intracranial Solitary Fibrous Tumor of the Skull Base: 2 Cases and Systematic Review of the Literature
Sricharan Gopakumar, Visish M. Srinivasan, Caroline C. Hadley, Adrish Anand, Marc Daou, Patrick J. Karas, Jacob Mandel, Shankar P. Gopinath, Akash J. Patel
World Neurosurgery.2021; 149: e345. CrossRef
Hemangiopericytoma/Solitary Fibrous Tumor in the central nervous system. Experience with surgery and radiotherapy as a complementary treatment: A 10-year analysis of a heterogeneous series in a single tertiary center
Pedro Miguel González-Vargas, José Luis Thenier-Villa, Pablo Sanromán Álvarez, Alexandre Serantes Combo, Lourdes Calero Félix, Raúl Alejandro Galárraga Campoverde, Eva Azevedo González, Álvaro Martín-Gallego, Rosa Martínez-Rolan, Adolfo de la Lama Zaragoz
Neurocirugía.2020; 31(1): 14. CrossRef
Hemangiopericytoma/Solitary Fibrous Tumor in the central nervous system. Experience with surgery and radiotherapy as a complementary treatment: A 10-year analysis of a heterogeneous series in a single tertiary center
Pedro Miguel González-Vargas, José Luis Thenier-Villa, Pablo Sanromán Álvarez, Alexandre Serantes Combo, Lourdes Calero Félix, Raúl Alejandro Galárraga Campoverde, Eva Azevedo González, Álvaro Martín-Gallego, Rosa Martínez-Rolan, Adolfo de la Lama Zaragoz
Neurocirugía (English Edition).2020; 31(1): 14. CrossRef
Solitary fibrous tumor/hemangiopericytoma: treatment results based on the 2016 WHO classification
Kyoung Su Sung, Ju Hyung Moon, Eui Hyun Kim, Seok-Gu Kang, Se Hoon Kim, Chang-Ok Suh, Sun Ho Kim, Kyu-Sung Lee, Won Seok Chang, Jong Hee Chang
Journal of Neurosurgery.2019; 130(2): 418. CrossRef
Grading of meningeal solitary fibrous tumors/hemangiopericytomas: analysis of the prognostic value of the Marseille Grading System in a cohort of 132 patients
Nicolas Macagno, Rob Vogels, Romain Appay, Carole Colin, Karima Mokhtari, Benno Küsters, Pieter Wesseling, Dominique Figarella‐Branger, Uta Flucke, Corinne Bouvier
Brain Pathology.2019; 29(1): 18. CrossRef
Solitary fibrous tumor of the pineal region with delayed ectopic intracranial metastasis: A case report and review of the literature
Yongjie Wang, Jingying Zhang, Qichang Liu, Fuyi Liu, Xiangdong Zhu, Jianmin Zhang
Medicine.2019; 98(21): e15737. CrossRef
Case report: neonatal giant forehead hemangiopericytoma with a 5-year follow-up
AiJun Peng, LiBing Zhang, Hai Zhao, LiangXue Zhou
Medicine.2019; 98(47): e17888. CrossRef
Liquid Biopsy in Rare Cancers: Lessons from Hemangiopericytoma
Chiara Nicolazzo, Luciano Colangelo, Alessandro Corsi, Guido Carpino, Angela Gradilone, Chiara Sonato, Cristina Raimondi, Eugenio Gaudio, Paola Gazzaniga, Walter Gianni
Analytical Cellular Pathology.2018; 2018: 1. CrossRef
Surveillance for metastatic hemangiopericytoma-solitary fibrous tumors-systematic literature review on incidence, predictors and diagnosis of extra-cranial disease
Tarini Ratneswaren, Florence Rosie Avila Hogg, Mathew Joseph Gallagher, Keyoumars Ashkan
Journal of Neuro-Oncology.2018; 138(3): 447. CrossRef
Intracranial Solitary Fibrous Tumor
Eveline Claus, Patrick Seynaeve, Jeroen Ceuppens, Alain Vanneste, Koenraad Verstraete
Journal of the Belgian Society of Radiology.2017;[Epub] CrossRef
Comparison and evaluation of risk factors for meningeal, pleural, and extrapleural solitary fibrous tumors: A clinicopathological study of 92 cases confirmed by STAT6 immunohistochemical staining
Ji Min Kim, Yoon-La Choi, Yu Jin Kim, Hyung Kyu Park
Pathology - Research and Practice.2017; 213(6): 619. CrossRef
Molecular Testing of Brain Tumor
Sung-Hye Park, Jaekyung Won, Seong-Ik Kim, Yujin Lee, Chul-Kee Park, Seung-Ki Kim, Seung-Hong Choi
Journal of Pathology and Translational Medicine.2017; 51(3): 205. CrossRef
Solitary fibrous tumour presenting with a single bone metastasis: report of six cases and literature review
Vittoria Colia, Salvatore Provenzano, Carlo Morosi, Paola Collini, Salvatore Lorenzo Renne, Paolo G. Dagrada, Claudia Sangalli, Angelo Paolo Dei Tos, Andrea Marrari, Paolo G. Casali, Silvia Stacchiotti
Clinical Sarcoma Research.2016;[Epub] CrossRef

Review

The Continuing Value of Ultrastructural Observation in Central Nervous System Neoplasms in Children: Na Rae Kim, Sung-Hye Park; J Pathol Transl Med. 2015;49(6):427-437. Published online October 13, 2015; DOI: https://doi.org/10.4132/jptm.2015.09.19

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Abstract PDF: Central nervous system (CNS) neoplasms are the second most common childhood malignancy after leukemia and the most common solid organ neoplasm in children. Diagnostic dilemmas with small specimens from CNS neoplasms are often the result of multifactorial etiologies such as frozen or fixation artifact, biopsy size, or lack of knowledge about rare or unfamiliar entities. Since the late 1950s, ultrastructural examination has been used in the diagnosis of CNS neoplasms, though it has largely been replaced by immunohistochemical and molecular cytogenetic studies. Nowadays, pathologic diagnosis of CNS neoplasms is achieved through intraoperative cytology, light microscopy, immunohistochemistry, and molecular cytogenetic results. However, the utility of electron microscopy (EM) in the final diagnosis of CNS neoplasms and investigation of its pathogenetic origin remains critical. Here, we reviewed the distinguishing ultrastructural features of pediatric CNS neoplasms and emphasize the continuing value of EM in the diagnosis of CNS neoplasms.

Original Article

Diagnostic Accuracy of Cerebrospinal Fluid (CSF) Cytology in Metastatic Tumors: An Analysis of Consecutive CSF Samples: Yoon Sung Bae, June-Won Cheong, Won Seok Chang, Sewha Kim, Eun Ji Oh, Se Hoon Kim; Korean J Pathol. 2013;47(6):563-568. Published online December 24, 2013; DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.6.563

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Abstract PDF

Background

Cerebrospinal fluid (CSF) examination can be used to verify the presence of primary malignancies as well as cases of central nervous system (CNS) metastasis. Because of its importance, there have been several studies concerning the sensitivity of CSF cytology. To determine the practical use and reproducibility of diagnoses based on CSF cytology, we evaluated this test by analyzing cytology results from consecutive CSF samples.

Methods

Between July 2010 and June 2013, 385 CSF cytology samples from 42 patients were collected. The samples were gathered using a ventricular catheter and reservoir. CSF cytology of all patients was examined more than two times with immunocytochemistry for cytokeratin.

Results

Primary neoplastic sites and histologic types of patients' metastatic cancer were diverse. The overall sensitivity for detecting malignancy was 41.3%. Even within short-term intervals, diagnoses frequently changed.

Conclusions

Our results were inconsistent, with low sensitivity, when compared to the results of previous studies. However, CSF evaluation can still provide valuable diagnostic and prognostic information because adjuvant treatments are now routinely performed in patients with CNS metastasis. Negative CSF cytology results should not be ignored, and continuous CSF follow-up is essential for following the clinical course of patients with metastatic cancer involving the CNS.

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Case Reports

Intracranial Fibromatosis: A Case Report.: Jeong Ju Lee, Jeoung Hun Kim, Shin Kwang Khang, Kyung Ja Cho, Jihun Kim; Korean J Pathol. 2011;45:S89-S92.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.S1.S89

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Abstract PDF

Fibromatosis can occur at various sites, but intracranial fibromatosis is exceptionally rare. Here, we report a case of intracranial fibromatosis arising in the suprasellar area of a 52-year-old woman who had undergone a surgery at that site. A computed tomography scan revealed a heavily calcified, highly enhancing, poorly demarcated mass in the left sellar area that extended into the left suprasellar, parasellar areas, and orbital apex and completely encased the left distal inferior cerebral artery. Histologic and immunohistochemical features were compatible with those of fibromatosis, although the cellularity was focally higher than usual. The etiology of extra-abdominal fibromatosis is unknown, but physical injuries such as trauma and irradiation have been reported to be associated with its occurrence. Although fibromatosis is rare in the intracranial area, it should be considered as a differential diagnosis when an intracranial mass occurs at a previously injured site.

Citations

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Infantile Intracranial Aggressive Fibromatosis: Report of Two Cases with a Review of the Literature
Baocheng Wang, Jie Ma, Huiming Jin
Pediatric Neurosurgery.2012; 48(3): 181. CrossRef

Malignant Rhabdoid Tumor of the Cerebellum in an Adult: A case report.: Young Min Kim, Jae Hee Suh, Tae Sook Kim, Shin Kwang Khang; Korean J Pathol. 1998;32(6):460-465.

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Abstract: Malignant rhabdoid tumor (MRT) is a rare and highly aggressive neoplasm of infancy and childhood. Although it was originally described and most frequently reported in the kidney, it may occur in various extra-renal sites such as the liver, thymus, and soft tissue. In the last decade primary central nervous system (CNS) MRTs have been reported in both the supra- and infratentorial compartments. Patients with CNS MRT were generally below the age of two and reports in adults are extremely rare. This is a case of primary cerebellar MRT in a 24-year-old woman, who had presented with intermittent headache, vocal cord palsy, and cerebellar dysfunctions such as abnormal finger to nose test and tandem gait. By magnetic resonance imaging scan, a well-enhancing solid mass was demonstrated at the posterior fossa filling the 4th ventricle, which extended into the medulla and cervical cord via the foramen of Magendie. Histologically, the monotonous polygonal tumor cells were arranged in diffuse sheet with occasional hemorrhagic necrosis. The nuclei were vesicular and eccentrically located due to eosinophilic, PAS-positive, intracytoplasmic inclusions with prominent nucleoli. They were diffusely or focally immunoreactive for vimentin, neurofilament, cytokeratin, GFAP, synaptophysin, and smooth muscle actin, while epithelial membrane antigen and desmin were negative. Ultrastructurally, the polyhedral tumor cells were densely packed with primitive intercellular junctions. Scanty fibrillar intermediate filaments were intermingled with cellular organelles. Postoperatively, craniospinal irradiation and systemic chemotherapy have been done and she has been free of tumor recurrence during the 13 months' follow-up periods.

Original Articles

Primary Central Nervous System Lymphomas; A Clinicopathologic Study of 18 Cases.: Yu Kyung Jeong, Young Hyeh Ko, Dong Kyu Na, Yeon Lim Suh, Sang Yong Song, Dae Shik Kim, Mi Kyung Kim, Howe Jung Ree; Korean J Pathol. 1998;32(9):670-679.

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Abstract: The incidence of a primary central nervous system (CNS) lymphoma in western countries is about 1% of all the intracranial tumors and has increased 2.2% over the last decade. A similar pattern of increased frequency is observed in Korea. Although most CNS lymphomas in western countries are high grade tumors carrying poor prognosis, the clinicopathologic features of the Korean CNS lymphoma have not been well studied. We report clinicopathological features of 18 cases of histologically proven primary brain lymphoma. The mean age of the patients was 50 years and there was no sex difference. The clinical and radiological characteristics included multiple site of occurrence, infrequent extracranial spread, and frequent seeding via cerebrospinal fluid. No patients were immune-compromised host. Of 18 cases, 15 cases were of B-lineage and 2 cases were of T-lineage. According to REAL classification, there were 12 cases of diffuse large B cell lymphoma, two cases of B cell lymphomas of small lymphoid cell, and two cases of peripheral T cell lymphoma, unspecified. The remaining subtypes were not subclassified because of inadequate material. Pleomorphic cytologic features and necrosis of varying extent were frequent in the cases of diffuse large B-cell lymphoma. These results suggest that overall clinicopathologic features of primary malignant lymphomas of the central nervous system in Korea are similar to those of western countries.

Prognostic Implications of Ki-67 Labelling Index and p53, bcl-2 Protein Expression in the Primary Central Nervous System Lymphoma.: Jae Ho Han, Woo Ick Yang, Tai Seung Kim; Korean J Pathol. 2000;34(6):456-464.

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Abstract PDF: It has been claimed that CNS lymphoma, a rare neoplasm accounting for only a small fraction of malignant brain tumors, occurs with increasing frequency in immunologically normal as well as immunocompromised individuals. We investigated the prognostic value of Ki-67 index, p53, and bcl-2 oncoprotein expression in relation to the clinicopathological parameters in the primary CNS lymphoma patients. The tumors were graded by Kiel classification and the Working formulation and included 33 high-grade, 4 intermediate-grade, and 5 low-grade lymphomas. The phenotype was determined in 38 cases: 30 were B cell type and 8 were T cell type. All cases displayed variable degrees of nuclear Ki-67 staining from 1.0% to 92.0% (mean 51.1%). A highly significant correlation was established between the proportion of Ki-67 positive cells and the classification into grades (p=0.0002) and phenotypes (p=0.0002). Overexpression of p53 and bcl-2 protein was found in 37.1% and 51.4% of 35 patients, respectively. And p53 expression was significantly increased in B cell type (p=0.02). On Kaplan-Meier survival curve, the phenotype, grade of tumors, and p53 and bcl-2 protein expression were not correlated with overall survival. On multivariate analyses, overall survival was independently influenced by Ki-67 index. In conclusion, it is suggested that Ki-67 proliferating index is the most important marker for predicting biologic behavior of the primary CNS lymphoma.

The Pattern of Cell Proliferation and Apoptosis in Human Embryonic and Fetal Brain.: Suk Jin Choi, Jung Ran Kim; Korean J Pathol. 2002;36(1):38-44.

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Abstract PDF: BACKGROUND
Cell proliferation and apoptosis account for the major morphogenetic mechanisms during development of the central nervous system. We investigated these processes in developing human brains.
METHODS
We examined human embryonic and fetal brains. Cell proliferation was analysed by classical histology and MIB-1 immunohistochemistry; cell death was investigated by the TdT-mediated dUTP-biotin nick end labelling method.
RESULTS
Most proliferating cells were observed in the ventricular zone (VZ) in the 3rd-10th week of gestational age (GA), and in both the VZ and the subventricular zone (SV) in the 19-24th week of GA. The proliferation index of the VZ was highest in the 8th week of GA and then decreased as the GA advanced. Apoptotic cells were observed in the VZ as early as the 5th week of GA. They were also observed in the intermediate zone in the 19-24th week of GA, although they were significantly lower in amount compared to that in the VZ and SV.
CONCLUSIONS
These results suggest that apoptosis occurring early in the embryonic period is related to a cellular mechanism which selects and determines the cells that are committed to migration and differentiation during the development of the human brain.

Cytology of Crush Preparation in Central Nervous System Lesion.: Young Il Yang, Sul Mi Park, Young Joo Kim, Shin Kwang Khang; Korean J Cytopathol. 1994;5(2):79-89.

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Abstract PDF: This study was performed in order to evaluate the accuracy and the usefulness of the cytology of crush preparation in central nervous system(CNS) lesions. Forty four intraoperative biopsies were performed at the time of craniotomy including 34 benign and 10 malignant lesions. Crush preparations were prepared from tiny tissue fragments of craiotomy products. All cases were stained with toluidine blue. Intraoperative diagnoses made on cytologic examination were compared with the final paraffin section diagnoses. Comparison between the results of the cytologic and histologic findings revealed an overall diagnostic accuracy of 88.6%. This study attests to the diagnostic accuracy of cytologic examination in CNS lesions. The detailed cytologic features are described and important criteria for the cytodiagnosis of CNS lesions are discussed.

Diagnostic Accuracy of Fine Needle Aspiration Cytology in Thyroid Lesions: Analysis of Histologically Confirmed 153 Cases.: Kyeong Mee Park, Ill Hyang Ko; Korean J Cytopathol. 1996;7(2):122-133.

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Abstract PDF: This is a retrospective review of fine-needle aspiration cytology(FNAC) smears of 153 cases of thyroid disease performed during August 1989 to July 1995, which were confirmed histologically following surgical operations. FNAC results showed 63 cases(41.2%) of adenomatous goiter, 45 cases(29.4%) of papillary carcinoma, 29 cases(19.0%) of follicular neoplasm, 4 cases(2.6%) of follicular variant of papillary carcinoma, 4 cases(2.6%) of Hashimoto's thyroiditis, 4 cases(2.6%) of Hurthle cell neoplasm, 2 cases(1.3%) of medullary carcinoma and one case(O.7%) each of subacute thyroiditis and of anaplastic carcinoma. The overall accuracy of cytological diagnosis was 83.7%. These data strongly suggest thyroid FNAC is a reliable preoperative diagnostic tool, but FNAC has been less valuable in the diagnosis of follicular lesions than any other disease of the thyroid. Adenomatous goiter was not infrequently interpreted as follicular neoplasia that requires surgery for diagnostic .conformation and vice versa. The following findings are considered to be compatible with follicular neoplasm: 1) microfolticles, 2) nuclear grooving, 3) irregularity of nuclear membrane, and 4) irregular arrangement or crowding of follicular cells in groups. The FNAC criteria of adenomatous goiter are as follows: 1) atrophic follicular cells, 2) presence of macrophages, 3) abundant colloid, and 4) large follicles. It is recommended that aspiration of thyroid lesions in order to analyse with critical clinico pathological approach and surgery is considered only for nodules that are clinically suspicious or unresponsive to hormone therapy or when a diagnosis of follicular neoplasm is made.

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