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Case Studies
Abrupt Dyskeratotic and Squamoid Cells in Poorly Differentiated Carcinoma: Case Study of Two Thoracic NUT Midline Carcinomas with Cytohistologic Correlation
Taebum Lee, Sangjoon Choi, Joungho Han, Yoon-La Choi, Kyungjong Lee
J Pathol Transl Med. 2018;52(5):349-353.   Published online July 27, 2018
DOI: https://doi.org/10.4132/jptm.2018.07.16
  • 8,778 View
  • 144 Download
  • 14 Web of Science
  • 14 Crossref
AbstractAbstract PDF
Cytologic diagnosis of nuclear protein in testis (NUT) midline carcinoma (NMC) is important due to its aggressive behavior and miserable prognosis. Early diagnosis of NMC can facilitate proper management, and here we report two rare cases of thoracic NMC with cytohistologic correlation. In aspiration cytology, the tumor presented with mixed cohesive clusters and dispersed single cells, diffuse background necrosis and many neutrophils. Most of the tumor cells had scanty cytoplasm and medium-sized irregular nuclei, which had fine to granular nuclear chromatin. Interestingly, a few dyskeratotic cells or squamoid cell clusters were present in each case. Biopsy specimen histology revealed more frequent squamous differentiation, and additional immunohistochemistry tests showed nuclear expression of NUT. Because this tumor has a notorious progression and has been previously underestimated in terms of its prevalence, awareness of characteristic findings and proper ancillary tests should be considered in all suspicious cases.

Citations

Citations to this article as recorded by  
  • Diagnostic significance and cytological features of NUT carcinoma by EBUS‐FNA, a case report and literature review
    Yaping Ju, Miriam Velazquez, Andy Sherrod, Tiannan Wang
    Cytopathology.2024; 35(4): 497.     CrossRef
  • Exploring cytologic features and potential diagnostic challenges of metastatic NUT carcinoma to the parotid gland: A case report and a comprehensive literature review
    Crystal Y. Li, Salih Salihoglu, Francisco J. Civantos, Jaylou M. Velez Torres
    Diagnostic Cytopathology.2024;[Epub]     CrossRef
  • Nuclear Protein in Testis (NUT) Carcinoma: A Comprehensive Immunohistochemical Analysis of 57 Cases With Consideration of Interpretation and Pitfall Recognition
    Ayesha Farooq, Allison L. Kerper, Jennifer M. Boland, Ying-Chun Lo
    Archives of Pathology & Laboratory Medicine.2024; 148(8): 898.     CrossRef
  • BRD3‐NUTM1‐expressing NUT carcinoma of lung on endobronchial ultrasound‐guided transbronchial needle aspiration cytology, a diagnostic pitfall
    Sameer Chhetri Aryal, Shereen Zia, Shannon Rodgers, Yulei Shen, Kyle Perry, Lisi Yuan
    Diagnostic Cytopathology.2022;[Epub]     CrossRef
  • Nuclear protein of the testis midline carcinoma of the thorax
    Ayae Saiki, Keita Sakamoto, Yuan Bee, Takehiro Izumo
    Japanese Journal of Clinical Oncology.2022; 52(6): 531.     CrossRef
  • Approach to Mediastinal Fine Needle Aspiration Cytology
    Zaibo Li, Huihong Xu, Fang Fan
    Advances in Anatomic Pathology.2022; 29(6): 337.     CrossRef
  • Diagnosis, Treatment and Prognosis of Primary Pulmonary NUT Carcinoma: A Literature Review
    Jiaqian Yuan, Zhili Xu, Yong Guo
    Current Oncology.2022; 29(10): 6807.     CrossRef
  • Case report: Immunovirotherapy as a novel add-on treatment in a patient with thoracic NUT carcinoma
    Linus D. Kloker, Branko Calukovic, Katrin Benzler, Alexander Golf, Sebastian Böhm, Sven Günther, Marius Horger, Simone Haas, Susanne Berchtold, Julia Beil, Mary E. Carter, Tina Ganzenmueller, Stephan Singer, Abbas Agaimy, Robert Stöhr, Arndt Hartmann, Tho
    Frontiers in Oncology.2022;[Epub]     CrossRef
  • Cytomorphology of primary pulmonary NUT carcinoma in different cytology preparations
    Rimlee Dutta, Aruna Nambirajan, Saurabh Mittal, Sinchita Roy‐Chowdhuri, Deepali Jain
    Cancer Cytopathology.2021; 129(1): 53.     CrossRef
  • Update on genetically defined lung neoplasms: NUT carcinoma and thoracic SMARCA4-deficient undifferentiated tumors
    Kyriakos Chatzopoulos, Jennifer M. Boland
    Virchows Archiv.2021; 478(1): 21.     CrossRef
  • Immunotherapy and Targeting the Tumor Microenvironment: Current Place and New Insights in Primary Pulmonary NUT Carcinoma
    Xiang Li, Hui Shi, Wei Zhang, Chong Bai, Miaoxia He, Na Ta, Haidong Huang, Yunye Ning, Chen Fang, Hao Qin, Yuchao Dong
    Frontiers in Oncology.2021;[Epub]     CrossRef
  • Prevalence of NUT carcinoma in head and neck: Analysis of 362 cases with literature review
    Taebum Lee, Junhun Cho, Chung‐Hwan Baek, Young‐Ik Son, Han‐Sin Jeong, Man Ki Chung, Sang Duk Hong, Yong Chan Ahn, Dong Ryul Oh, Jae Myoung Noh, Keunchil Park, Myung‐Ju Ahn, Hyung‐Jin Kim, Yi Kyung Kim, Young Hyeh Ko
    Head & Neck.2020; 42(5): 924.     CrossRef
  • Lung nuclear protein in testis carcinoma in an elderly Korean woman: A case report with cytohistological analysis
    Hwa Jin Cho, Hyun‐Kyung Lee
    Thoracic Cancer.2020; 11(6): 1724.     CrossRef
  • Clinicopathological characteristics of primary lung nuclear protein in testis carcinoma: A single‐institute experience of 10 cases
    Yoon Ah Cho, Yoon‐La Choi, Inwoo Hwang, Kyungjong Lee, Jong Ho Cho, Joungho Han
    Thoracic Cancer.2020; 11(11): 3205.     CrossRef
Malignant Pleural Effusion from Metastatic Prostate Cancer: A Case Report with Unusual Cytologic Findings
Jinyoung Jeon, Tae-Jung Kim, Hong Sik Park, Kyo-Young Lee
J Pathol Transl Med. 2018;52(4):257-261.   Published online June 7, 2018
DOI: https://doi.org/10.4132/jptm.2018.05.08
  • 9,517 View
  • 126 Download
  • 3 Web of Science
  • 5 Crossref
AbstractAbstract PDF
We present a case of 55-year-old man who complained of dyspnea and sputum for a month. He was an ex-smoker with a history of prostate cancer and pulmonary tuberculosis. Chest radiographs revealed bilateral pleural effusions of a small to moderate amount. Pigtail catheters were inserted for drainage. The pleural fluid consisted of large clusters and tightly cohesive groups of malignant cells, which however could not be ascribed to prostate cancer with certainty. We performed immunocytochemical panel studies to determine the origin of cancer metastasis. The immunostaining results were positive for prostate-specific antigen, alpha-methylacyl-coenzyme A racemase, and Nkx 3.1, consistent with prostate cancer. Pleural effusion associated with prostate cancer is rare. To our knowledge, this is the first case report in Korea to describe cytologic features of malignant pleural effusion associated with prostate cancer.

Citations

Citations to this article as recorded by  
  • Pleural Metastasis as an Initial Presentation of Prostate Cancer: Case Report and Literature Review
    Katarzyna Skrobisz, Kevin Miszewski, Laura Miszewska, Michał Bieńkowski, Marcin Matuszewski, Michał Studniarek
    Diagnostics.2025; 15(6): 666.     CrossRef
  • EBUS-TBNA pleural biopsy reveals prostate cancer metastasis: A rare case report and review of the literature
    Fotios Sampsonas, Dimitrios Komninos, Vasilina Sotiropoulou, Matthaios Katsaras, Dimitra Gkanetsou, Ourania Papaioannou, Panagiota Tsiri, Vasiliki Tzelepi, Argyrios Tzouvelekis
    Pneumon.2024; 37(2): 1.     CrossRef
  • Cytopathological Features of Extensive Bilateral Pleural Effusions in Metastatic Prostate Cancer: Report of a Rare Case
    Hehua Huang, Caroline Yap
    Cureus.2024;[Epub]     CrossRef
  • Bilateral pleural effusion: etiology, diagnostics
    N. A. Stogova
    PULMONOLOGIYA.2022; 32(6): 885.     CrossRef
  • Rare Metastatic Prostate Cancer Mimicking Lymphoma with Malignant Pleural Effusion
    Tung Liu, En Meng, Yu-Chun Lin, Tai-Kuang Chao, Yi-Ming Chang
    Journal of Medical Sciences.2021; 42(1): 46.     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
  • 11,177 View
  • 116 Download
  • 21 Web of Science
  • 18 Crossref
AbstractAbstract 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

Citations to this article as recorded by  
  • 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
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    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
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    AiJun Peng, LiBing Zhang, Hai Zhao, LiangXue Zhou
    Medicine.2019; 98(47): e17888.     CrossRef
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    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
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    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
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Case Study
Human Herpesvirus 8-Negative and Epstein-Barr Virus-Positive Effusion-Based Lymphoma in a Patient with Human Immunodeficiency Virus
Jung-Woo Choi, Younghye Kim, Ju-Han Lee, Young-Sik Kim
J Pathol Transl Med. 2015;49(5):409-412.   Published online June 17, 2015
DOI: https://doi.org/10.4132/jptm.2015.06.03
  • 8,619 View
  • 65 Download
  • 3 Web of Science
  • 3 Crossref
AbstractAbstract PDF
A 39-year-old man infected with human immunodeficiency virus (HIV) was admitted to our hospital because of sudden onset of chest pain. Chest radiography revealed pneumothorax of the right lung. Computed tomographic scans disclosed a 5.8-cm-sized emphysematous bulla in the right middle lobe of the lung. Histologically, the wedge-resected lung showed medium to large atypical cells within the bullous cavity of the Pneumocystis jirovecii pneumonia, without solid mass formation. These atypical cells were confirmed to be large B-cell lymphoma, Epstein-Barr virus–positive and human herpesvirus 8–negative. Therefore, this case was not diagnosed as primary effusion lymphoma, but effusion-based lymphoma arising in an emphysematous cavity of an HIV-infected patient. This type of effusion-based lymphoma has never been reported, and, although rare, it should be noted in order to clinically diagnose this lymphoma.

Citations

Citations to this article as recorded by  
  • Primary Effusion Lymphoma: A Timely Review on the Association with HIV, HHV8, and EBV
    Chih-Yi Liu, Bo-Jung Chen, Shih-Sung Chuang
    Diagnostics.2022; 12(3): 713.     CrossRef
  • Human herpesvirus 8-negative effusion-based large B-cell lymphoma: a distinct entity with unique clinicopathologic characteristics
    Savanah D. Gisriel, Ji Yuan, Ryan C. Braunberger, Danielle L.V. Maracaja, Xueyan Chen, Xiaojun Wu, Jenna McCracken, Mingyi Chen, Yi Xie, Laura E. Brown, Peng Li, Yi Zhou, Tarsheen Sethi, Austin McHenry, Ronald G. Hauser, Nathan Paulson, Haiming Tang, Eric
    Modern Pathology.2022; 35(10): 1411.     CrossRef
  • Age and CD20 Expression Are Significant Prognostic Factors in Human Herpes Virus-8-negative Effusion-based Lymphoma
    Tomomi Kubota, Yosuke Sasaki, Eisuke Shiozawa, Masafumi Takimoto, Tsunekazu Hishima, Ja-Mun Chong
    American Journal of Surgical Pathology.2018; 42(12): 1607.     CrossRef
Review
Current Concepts in Primary Effusion Lymphoma and Other Effusion-Based Lymphomas
Yoonjung Kim, Chan Jeong Park, Jin Roh, Jooryung Huh
Korean J Pathol. 2014;48(2):81-90.   Published online April 28, 2014
DOI: https://doi.org/10.4132/KoreanJPathol.2014.48.2.81
  • 13,283 View
  • 163 Download
  • 29 Crossref
AbstractAbstract PDF

Primary effusion lymphoma (PEL) is a human herpes virus 8 (HHV8)-positive large B-cell neoplasm that presents as an effusion with no detectable tumor in individuals with human immunodeficiency virus infection or other immune deficiencies. PEL is an aggressive neoplasm with a poor prognosis. PEL cells show diverse morphologies, ranging from immunoblastic or plasmablastic to anaplastic. The immunophenotype of PEL is distinct, but its lineage can be misdiagnosed if not assessed thoroughly. PEL cells usually express CD45, lack B- and T-cell-associated antigens, and characteristically express lymphocyte activation antigens and plasma cell-associated antigens. Diagnosis of PEL often requires the demonstration of a B-cell genotype. HHV8 must be detected in cells to diagnose PEL. In most cases, PEL cells also harbor the Epstein-Barr virus (EBV) genome. Similar conditions associated with HHV8 but not effusion-based are called "extracavitary PELs." PELs should be differentiated from HHV8-negative, EBV-positive, body cavity-based lymphomas in patients with long-standing chronic inflammation; the latter can occur in tuberculous pleuritis, artificial pneumothorax, chronic liver disease and various other conditions. Despite their morphological similarity, these various lymphomas require different therapeutic strategies and have different prognostic implications. Correct diagnosis is essential to manage and predict the outcome of patients with PEL and related disorders.

Citations

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Case Study
Myxoid Liposarcoma with Cartilaginous Differentiation: A Case Study with Cytogenetical Analysis
Hyunchul Kim, Won Hwangbo, Sangjeong Ahn, Suhjin Kim, Insun Kim, Chul Hwan Kim
Korean J Pathol. 2013;47(3):284-288.   Published online June 25, 2013
DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.3.284
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AbstractAbstract PDF

Myxoid liposarcoma is a subtype of liposarcoma. This specific subtype can be identified based on its characteristic histological and cytogenetical features. The tumor has a fusion transcript of the CHOP and TLS genes, which is caused by t(12;16)(q13;p11). Most of the fusion transcripts that have been identified fall into three categories, specifically type I (exons 7-2), type II (exons 5-2), and type III (exons 8-2). A total of seven myxoid liposarcomas associated with the rare phenomenon of cartilaginous differentiation have been documented in the literature. Currently, only one of these cases has been cytogenetically analyzed, and the analysis indicated that it was a type II TLS-CHOP fusion transcript in both the typical myxoid liposarcoma and cartilaginous areas. This study presents a second report of myxoid liposarcoma with cartilaginous differentiation, and includes a cytogenetical analysis of both the myxoid and cartilaginous areas.

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Original Articles
Finding and Characterizing Mammary Analogue Secretory Carcinoma of the Salivary Gland
Min Jung Jung, Joon Seon Song, Sang Yoon Kim, Soon Yuhl Nam, Jong-Lyel Roh, Seung-Ho Choi, Sung-Bae Kim, Kyung-Ja Cho
Korean J Pathol. 2013;47(1):36-43.   Published online February 25, 2013
DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.1.36
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AbstractAbstract PDF
Background

A new tumor entity of the salivary glands, mammary analogue secretory carcinoma (MASC) with ETV6-NTRK3 translocation, has recently been proposed. MASC was originally diagnosed as adenocarcinoma, not otherwise specified (ANOS), or acinic cell carcinoma (AciCC) by the current World Health Organization classification. We aimed to identify MASC cases by molecular tests, and to characterize their clinical, histological, and immunohistochemical features.

Methods

Thirty cases of MASC candidates were selected after review of 196 salivary gland tumors, and subjected to break-apart ETV6 fluorescence in situ hybridization (FISH), and immunohistochemical study for S100 protein, gross cystic disease fluid protein 15, DOG1, estrogen receptor, and progesterone receptor.

Results

Valid FISH results were obtained in 23 cases, and 13 positive cases were retrieved. MASCs were histologically varied, and the most frequent features observed in 10 cases were low-grade papillary/cystic/glandular patterns, intraluminal secretory materials, ovoid/wrinkled nuclei, and relatively abundant granular eosinophilic cytoplasms, corresponding to papillary-cystic or follicular types of AciCC. All cases showed diffuse immunopositivity for S100 protein. Three cases developed recurrences, but all patients remained alive.

Conclusions

MASC could be a molecularly well-defined salivary gland neoplasm, encompassing some portions of AciCC and ANOS, but its histological spectrum and clinical implication require further investigation.

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Diagnostic Utility of the JAZF1/JJAZ1 Gene Fusion in Endometrial Stromal Sarcomas and Their Histologic Variants.
Sang Ryung Lee, Joon Seon Song, Ga Hye Kim, Jene Choi, Hyung Kyoung Kim, Yonghee Lee, Kyu Rae Kim
Korean J Pathol. 2011;45(5):498-505.
DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.5.498
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AbstractAbstract PDF
BACKGROUND
The diagnosis of endometrial stromal sarcoma (ESS) is often difficult in cases showing diverse histological differentiation or in undifferentiated endometrial sarcoma (UES). Recently, JAZF1/JJAZ1 gene fusion has been described as a defining feature of low-grade ESS (LGESS). However, its prevalence is variably reported, and the diagnostic utility has rarely been examined for cases showing various histological differentiation.
METHODS
To test the diagnostic utility of JAZF1/JJAZ1 gene fusion in difficult cases, we compared the prevalence of the JAZF1/JJAZ1 fusion gene in LGESS with and without histological differentiation.
RESULTS
The JAZF1/JJAZ1 fusion transcript was detected in 18 of 21 LGESS (85.7%), including 14 classical LGESS (93%), four LGESS with diverse histological differentiation (67%), and two with UES (28.6%). Positive cases included two LGESS with sex cord-like differentiation, one with osseous differentiation, and two UES. LGESS showing smooth muscle differentiation revealed the fusion transcript only in the classic area. Direct sequencing analysis of two LGESS revealed a previously reported breakpoint at t(7;17)(p15;q21).
CONCLUSIONS
The JAZF1/JJAZ1 fusion gene was identified in a significant proportion of LGESS showing secondary histological differentiation except in cases with smooth muscle differentiation. Thus, this fusion gene may be useful to confirm the diagnosis in difficult cases of LGESS.
Case Reports
Adrenal Cortical Adenoma Developed in Adrenohepatic Fusion, a Mimicry of Hepatocellular Carcinoma: A Case Report.
Sun A Kim, Young Joo Lee, Kyoung Won Kim, Gyungyub Gong
Korean J Pathol. 2011;45(2):196-200.
DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.2.196
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AbstractAbstract PDF
Adrenohepatic fusion is the union of the liver and adrenal gland with close intermingling of their respective parenchymal cells. Adrenal cortical adenoma arising in adrenohepatic fusion tissue is extremely rare, although adrenohepatic fusion itself is relatively common. Here we report a case of a 59-year-old man with a mass in the right lobe of his liver. The mass showed slight hyperattenuation during arterial phase and hypoattenuation during portal phase on dynamic computed tomography with contrast enhancement. On pathology, the mass consisted of round to polygonal cells with clear microvesicular or eosinophilic cytoplasm, arranged in nests or in a trabecular pattern. The tumor cells were positive for inhibin and melan-A, but negative for Hep Par-1. In the periphery of the mass, adrenohepatic fusion was identified between the liver and adrenal gland, and was simultaneously resected with the mass. We report this rare case, and discuss its clinical implications, especially the differential diagnosis with hepatocellular carcinoma.

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  • Adrenal cortical adenoma arising in an adreno-hepatic fusion: Case report and literature review of a potential diagnostic pitfall
    Adam Stenman, Ivan Shabo, Jan Zedenius, C. Christofer Juhlin
    Human Pathology Reports.2022; 29: 300656.     CrossRef
  • Intrahepatic adrenocortical adenoma arising from adrenohepatic fusion mimicking hepatic malignancy
    Yong Soo Cho, Jin Woong Kim, Hyun Ju Seon, Ju-Yeon Cho, Jun-Hee Park, Hyung Joong Kim, Yoo Duk Choi, Young Hoe Hur
    Medicine.2019; 98(23): e15901.     CrossRef
  • Direct and indirect imaging features of adrenohepatic fusion
    Jung Jae Park, Byung Kwan Park, Chan Kyo Kim
    Abdominal Radiology.2016; 41(2): 377.     CrossRef
Cytologic Diagnosis of Malignant Pleural Effusion in Multiple Myeloma: Two Case Reports.
Yoo Duk Choi, Sung Sun Kim, Chang Woo Han, Ji Shin Lee, Jong Hee Nam, Sang Woo Juhng, Chan Choi
Korean J Pathol. 2009;43(4):382-385.
DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.4.382
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AbstractAbstract PDF
Malignant pleural effusion in multiple myeloma (MM) is extremely rare and is associated with poor prognosis. We experienced two cases of MM IgA type with malignant pleural effusion. The diagnoses were based on characteristic cytology and CD138 immunocytochemistry. The patients received several cycles of combination chemotherapy, since symptoms were more aggressive with an uncontrolled pleural effusion. We review the clinical features of these cases and literature concerning myelomatous pleural effusion.

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  • Características de los pacientes con derrame pleural mielomatoso. Revisión sistemática
    V. Riveiro, L. Ferreiro, M.E. Toubes, A. Lama, J.M. Álvarez-Dobaño, L. Valdés
    Revista Clínica Española.2018; 218(2): 89.     CrossRef
  • Characteristics of patients with myelomatous pleural effusion. A systematic review
    V. Riveiro, L. Ferreiro, M.E. Toubes, A. Lama, J.M. Álvarez-Dobaño, L. Valdés
    Revista Clínica Española (English Edition).2018; 218(2): 89.     CrossRef
  • A 76-Year-Old Man With Anemia, Bone Pain, and Progressive Dyspnea
    Thitiporn Suwatanapongched, Prapaporn Pornsuriyasak, Wasana Kanoksil, Thotsaporn Morasert, Warapat Virayavanich
    Chest.2014; 145(4): 913.     CrossRef
The Cytology of Metastatic Angiosarcoma in Pleural Fluid : A Case Report.
Na Rae Kim, Dong Hae Chung, Hyun Yee Cho
Korean J Pathol. 2009;43(3):285-259.
DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.3.285
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  • 51 Download
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AbstractAbstract PDF
A 74-year-old woman presented with an abrupt onset of dyspnea that she had experienced for a week. She had been suffering from cutaneous nodules in the scalp for a year. Thoracentesis of the pleural fluid was performed. The Papanicolaou-stained smears, Thin prep and cell block preparations revealed clusters of oval-shaped cells concentrically layered about amorphous acellular cores, i.e., there was microacinar lumen formation as well as singly scattered atypical cells. The cells occasionally demonstrated intracytoplasmic vacuoles and hemosiderin deposits. Those cells stained for CD31 and they were negative for pancytokeratin. Punch biopsy from the scalp nodules revealed angiosarcoma. There are currently few reported cases of angiosarcoma in an exfoliative pleural effusion. Angiosarcoma has diverse, heterogeneous cytologic features. Making the cytologic diagnosis of metastatic angiosarcoma in pleural fluid is a challenge for pathologists. Knowledge of the clinical history is of great help for diagnosing this tumor when it appears in rare sites. Immunopanels with CD31, pancytokeratin and TTF-1 are helpful for making the differential diagnosis. The pathologists should look for clues suggesting the presence of vascular differentiation in the exfoliative cytologic materials when a diagnosis of angiosarcoma is suspected.

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  • Metastatic pleural angiosarcoma: A diagnostic pitfall might be overcome by morphologic clues and clinical correlation
    Ok Ran Shin, Uiju Cho, Eundeok Chang, Kyung Jin Seo
    Diagnostic Cytopathology.2015; 43(8): 669.     CrossRef
  • Pleural fluid cytology in metastatic uterine angiosarcoma—A case report—
    Shiho KURODA, Shioto SUZUKI, Akira KURITA, Mari MURAKI, Fumihiko TANIOKA
    The Journal of the Japanese Society of Clinical Cytology.2014; 53(6): 498.     CrossRef
  • Intranasal delivery of biologics to the central nervous system
    Jeffrey J. Lochhead, Robert G. Thorne
    Advanced Drug Delivery Reviews.2012; 64(7): 614.     CrossRef
  • Fine Needle Aspiration Cytologic Findings of Angiosarcoma - Report of Two Cases -
    Jin Xian Ji, Young Chae Chu, Lucia Kim, Suk Jin Choi, In Suh Park, Jee Young Han, Joon Mee Kim, Kyu Ho Kim, Ju Young Song
    The Korean Journal of Pathology.2011; 45(2): 217.     CrossRef
The Cytologic Features of Desmoplastic Small Round Cell Tumor with Intranuclear Inclusions : A Case Report .
Ho Chang Lee, Hye Suk Han, Ok Jun Lee
Korean J Pathol. 2009;43(3):279-284.
DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.3.279
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AbstractAbstract PDF
Desmoplastic small round cell tumor (DSRCT) is a rare neoplasm of young adults and it is characterized by polyphenotypic differentiation. We experienced a case of abdominal DSRCT that occurred in a 19-year-old female who presented with painful swelling of her right forearm. The tumor was cytokeratin-negative and it exhibited some tumor cells with intranuclear inclusions. Molecular demonstration of EWS-WT1 fusion transcripts is particularly useful to confirm the diagnosis of DSRCT without epithelial differentiation. We report here on a case of cytokeratin-negative DSRCT that showed an unusual feature of intranuclear inclusions.
Primary Synovial Sarcoma of the Kidney: A Case Report and Literature Review.
Mee Ja Park, Tae Hwa Baek, Joo Heon Kim, Dong Wook Kang, Hye Kyung Lee, Hyun Jin Son
Korean J Pathol. 2009;43(3):274-278.
DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.3.274
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AbstractAbstract PDF
Synovial sarcoma is a rare renal neoplasm that is not easy to diagnose unless SYT-SSX fusion transcripts are identified. We report here on a case of primary renal synovial sarcoma in a 35-year-old woman. A mass was discovered by accident in the lower part of the right kidney when ultrasonography was performed, and it was removed via radical nephrectomy. Grossly, the tumor was a homogeneously tan-brown soft mass that measured 4.5x3.2x3.0 cm, and it was encircled by a well-defined cystic space. The lesion exhibited hypercellularity of the oval or short spindle cells that were arranged in various solid sheets or intersecting fascicles. Immunohistochemically, the tumor showed diffuse positivity for vimentin, bcl-2 and CD99, and it showed focal positivity for epithelial membrane antigen. The SYT-SSX fusion transcripts were detected by reverse transcription-polymerase chain reaction (RT-PCR). Synovial sarcoma should be considered in the differential diagnosis when a spindle cell neoplasm is encountered in the kidney.

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  • Primary Renal Synovial Sarcoma and Clinical and Pathological Findings: a Systematic Review
    Leandro Blas, Javier Roberti
    Current Urology Reports.2021;[Epub]     CrossRef
  • Primary Renal Synovial Sarcoma - A rare histology
    Premkumar Krishnappa, Mohan keshavamurthy, Shakir Tabrez, Sreeharsha Harinatha, Mohan Balaiah Aswathaiya
    Urology Case Reports.2020; 33: 101402.     CrossRef
Desmoplastic Small Round Cell Tumor with Ovarian Involvement: A Case Report.
Sang Hwa Lee, Wan Seop Kim, Ji Hoon Kim, Hye Seung Han, So Dug Lim, Sang Yoon Kim, Tae Sook Hwang
Korean J Pathol. 2009;43(2):185-188.
DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.2.185
  • 2,988 View
  • 34 Download
  • 1 Crossref
AbstractAbstract PDF
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm that preferentially involves the abdominal and pelvic cavities in relatively young males. We present a rare case of DSRCT arising in the ovary of a 16-year-old girl. During surgery, a 15 cm-sized huge mass was noted in the right ovary and wide spreading of the tumor was identified in the left ovary, uterine wall, and omentum and bowel wall. Histological investigation showed nests of small round cells with round nuclei and scanty eosinophilic cytoplasm accompanied with dense desmoplastic stroma. The immunohistochemistry showed that the tumor coexpressed epithelial, mesenchymal, and neuronal markers. The tumor cells ultrastructurally showed poorly developed cell junctions and occasionally showed intracytoplasmic aggregates of intermediate filaments. Molecular analysis of the tumor revealed chromosomal translocation t(11:22)(p13;q12) associated with the EWS-WT1 fusion protein. DSRCT should be included in the differential diagnosis of ovarian neoplasms in young patients.

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  • Desmoplastic small round cell tumor of the ovary
    Ravi Hari Phulware, Maitrayee Roy, Neeta Singh, Sunesh Kumar, Sandeep R. Mathur
    Indian Journal of Pathology and Microbiology.2021; 64(1): 206.     CrossRef
Original Article
Clinicopathologic Study of Chromosomal Aberrations in Gastric Lymphomas of Korean Patients.
Wook Youn Kim, Jung Ho Kim, Hyoungsuk Ko, Young A Kim, Yoon Kyung Jeon, Chul Woo Kim
Korean J Pathol. 2009;43(1):5-12.
DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.1.5
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AbstractAbstract PDF
BACKGROUND
The incidence and clinical correlation of MALT1 translocation and numerical aberrations in Korean gastric MALT lymphoma patients have been rarely reported. We studied the incidence and clinicopathologic relationship of these chromosomal aberrations in Korean gastric lymphomas.
METHODS
Seventy-six gastric lymphomas, which consisted of 40 low grade MALT lymphoma, 4 high grade MALT lymphoma and 32 diffuse large B-cell lymphoma (DLBCL) cases, were analyzed for the detection of t(11;18) API2-MALT1, t(14;18) IgH-MALT1 and aneuploidies of chromosomes 3 or 18 using fluorescence in situ hybridization.
RESULTS
The t(11;18) was demonstrated in 3 low grade MALT lymphomas (7.5%) and one DLBCL, which was associated with advanced stage, deeper invasion, and disease progression or relapse. The t(14;18) was demonstrated in none of these cases. Trisomy 3 and 18 were detected in 8 (11%) and 11 of 76 cases (12.5%) respectively, and found only in translocation-negative cases. Two of 4 high grade MALT lymphomas showed trisomy 18. All patients survived with successful second treatment after progression or relapse.
CONCLUSIONS
The t(11;18) API2-MALT1 was not quite frequent in Korean low grade gastric MALT lymphomas and was associated with advanced clinical situations. Overall prognosis was good for long-term follow-up regardless of progression or relapse.

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  • Clinicopathologic Study of Chromosomal Aberrations in Ocular Adnexal Lymphomas of Korean Patients
    Hokyung Choung, Young A Kim, Namju Kim, Min Joung Lee, Sang In Khwarg
    Korean Journal of Ophthalmology.2015; 29(5): 285.     CrossRef

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