Journal of Pathology and Translational Medicine

Case Studies

Primary pulmonary epithelioid inflammatory myofibroblastic sarcoma: a rare entity and a literature review: Priyanka Singh, Aruna Nambirajan, Manish Kumar Gaur, Rahul Raj, Sunil Kumar, Prabhat Singh Malik, Deepali Jain; J Pathol Transl Med. 2022;56(4):231-237. Published online July 7, 2022; DOI: https://doi.org/10.4132/jptm.2022.05.08

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Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is an aggressive subtype of inflammatory myofibroblastic tumor (IMT) harboring anaplastic lymphoma kinase (ALK) gene fusions and is associated with high risk of local recurrence and poor prognosis. Herein, we present a young, non-smoking male who presented with complaints of cough and dyspnoea and was found to harbor a large right lower lobe lung mass. Biopsy showed a high-grade epithelioid to rhabdoid tumor with ALK and desmin protein expression. The patient initially received 5 cycles of crizotinib and remained stable for 1 year; however, he then developed multiple bony metastases, for which complete surgical resection was performed. Histopathology confirmed the diagnosis of EIMS, with ALK gene rearrangement demonstrated by fluorescence in situ hybridization. Postoperatively, the patient is asymptomatic with stable metastatic disease on crizotinib and has been started on palliative radiotherapy. EIMS is a very rare subtype of IMT that needs to be included in the differential diagnosis of ALKexpressing lung malignancies in young adults.

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Mediastinal epithelioid inflammatory myofibroblastic sarcoma with the EML4‐ALK fusion: A case report and literature review
Tingyu Pan, Xinyu Sun, Xiao Wu, Futing Tang, Xianmei Zhou, Qian Wang, Shi Chen
Respirology Case Reports.2024;[Epub] CrossRef
Case report: Epithelioid inflammatory myofibroblastic sarcoma treated with an ALK TKI ensartinib
Mengmeng Li, Ruyue Xing, Jiuyan Huang, Chao Shi, Chunhua Wei, Huijuan Wang
Frontiers in Oncology.2023;[Epub] CrossRef
Epithelioid Inflammatory Myofibroblastic Sarcoma With Poor Response to Crizotinib: A Case Report
Soheila Aminimoghaddam, Roghayeh Pourali
Clinical Medicine Insights: Case Reports.2023;[Epub] CrossRef
Epithelioid inflammatory myofibroblastic sarcoma: a case report and brief literature review
Weidong Dou, Yu Guan, Tao Liu, Hang Zheng, Shuo Feng, Yingchao Wu, Xin Wang, Zhanbing Liu
Frontiers in Oncology.2023;[Epub] CrossRef

Rosette-forming epithelioid osteosarcoma in the rib: a rare case of location and morphology: Sun-Ju Oh; J Pathol Transl Med. 2021;55(6):406-409. Published online August 3, 2021; DOI: https://doi.org/10.4132/jptm.2021.06.22

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Abstract PDF: The rib is an unusual location for osteosarcoma and is reported in only 2% of all cases. The major histological variants of osteosarcoma are osteoblastic, chondroblastic, and fibroblastic, with a few rare variants including one epithelioid type. This report describes a 44-year-old male with an osteolytic mass in the right seventh rib. Histological examination revealed osteosarcoma with unique features of epithelioid appearance and rosette structures. To the best of our knowledge, this is the first reported case of a rosette-forming osteosarcoma of the rib that showed epithelioid morphology. Despite successful surgery, the patient’s prognosis was poor because this malignancy had an unusual location within the axial skeleton and was a rare histological variant.

TFE3-Expressing Perivascular Epithelioid Cell Tumor of the Breast: Hyunjin Kim, Jimin Kim, Se Kyung Lee, Eun Yoon Cho, Soo Youn Cho; J Pathol Transl Med. 2019;53(1):62-65. Published online October 1, 2018; DOI: https://doi.org/10.4132/jptm.2018.08.30

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Perivascular epithelioid cell tumor (PEComa) is a very rare mesenchymal tumor with a distinctive morphology and immunophenotype. PEComas usually harbor TSC2 alterations, although TFE3 translocations, which occur in MiT family translocation renal cell carcinoma and alveolar soft part sarcoma, are also possible. We recently experienced a case of PEComa with TFE3 expression arising in the breast. An 18-year-old female patient presented with a right breast mass. Histologically, the tumor consisted of epithelioid cells with alveolar structure and showed a diffuse strong expression of HMB45 and TFE3. TSC2 was preserved. Melan A and smooth muscle actin were negative. To our knowledge, this is the first Korean case of PEComa of the breast that intriguingly presented with TFE3 expression.

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Malignant lung PEComa (clear cell tumor): rare case report and literature review
Marcos Adriano Garcia Campos, Lucas Fernandes Vasques, Rafael Goulart de Medeiros, Érico Murilo Monteiro Cutrim, Ana Júlia Favarin, Sarah Rebecca Machado Silva, Gyl Eanes Barros Silva, Marcelo Padovani de Toledo Moraes, Mariana Lopes Zanatta, Diego Aparec
Frontiers in Oncology.2023;[Epub] CrossRef
Cathepsin K: A Versatile Potential Biomarker and Therapeutic Target for Various Cancers
Die Qian, Lisha He, Qing Zhang, Wenqing Li, Dandan Tang, Chunjie Wu, Fei Yang, Ke Li, Hong Zhang
Current Oncology.2022; 29(8): 5963. CrossRef
Endometrioid Carcinomas of the Ovaries and Endometrium Involving Endocervical Polyps: Comprehensive Clinicopathological Analyses
Jihee Sohn, Yurimi Lee, Hyun-Soo Kim
Diagnostics.2022; 12(10): 2339. CrossRef
Serous Carcinoma of the Endometrium with Mesonephric-Like Differentiation Initially Misdiagnosed as Uterine Mesonephric-Like Adenocarcinoma: A Case Report with Emphasis on the Immunostaining and the Identification of Splice Site TP53 Mutation
Sangjoon Choi, Yoon Yang Jung, Hyun-Soo Kim
Diagnostics.2021; 11(4): 717. CrossRef
Mesonephric-like Differentiation of Endometrial Endometrioid Carcinoma: Clinicopathological and Molecular Characteristics Distinct from Those of Uterine Mesonephric-like Adenocarcinoma
Sujin Park, Go Eun Bae, Jiyoung Kim, Hyun-Soo Kim
Diagnostics.2021; 11(8): 1450. CrossRef
Mesonephric-like Adenocarcinoma of the Uterine Corpus: Comprehensive Immunohistochemical Analyses Using Markers for Mesonephric, Endometrioid and Serous Tumors
Hyunjin Kim, Kiyong Na, Go Eun Bae, Hyun-Soo Kim
Diagnostics.2021; 11(11): 2042. CrossRef
Invasive Lobular Carcinoma With Extensive Clear Cells: A Pitfall in Diagnosis
Mark H. Kavesh, Daniel Sanchez, Jaya Ruth Asirvatham
International Journal of Surgical Pathology.2020; 28(2): 169. CrossRef
Glycogen-rich Clear Cell Carcinoma of the Breast: A Comprehensive Review
Semir Vranic, Faruk Skenderi, Vanesa Beslagic, Zoran Gatalica
Applied Immunohistochemistry & Molecular Morphology.2020; 28(9): 655. CrossRef
TFE3-expressing primary perivascular epithelioid cell tumor of the Lymph node mimicking nodal relapse of rectal cancer: A case report
Jongmin Park, An Na Seo
International Journal of Surgery Case Reports.2019; 59: 46. CrossRef

Perivascular Epithelioid Cell Tumor in the Stomach: Sun Ah Shin, Jiwoon Choi, Kyung Chul Moon, Woo Ho Kim; J Pathol Transl Med. 2017;51(4):428-432. Published online April 4, 2017; DOI: https://doi.org/10.4132/jptm.2016.09.16

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Abstract PDF

Perivascular epithelioid cell tumors or PEComas can arise in any location in the body. However, a limited number of cases of gastric PEComa have been reported. We present two cases of gastric PEComas. The first case involved a 62-year-old woman who presented with a 4.2 cm gastric subepithelial mass in the prepyloric antrum, and the second case involved a 67-year-old man with a 5.0 cm mass slightly below the gastroesophageal junction. Microscopic examination revealed that both tumors were composed of perivascular epithelioid cells that were immunoreactive for melanocytic and smooth muscle markers. Prior to surgery, the clinical impression of both tumors was gastrointestinal stromal tumor (GIST), and the second case was erroneously diagnosed as GIST even after microscopic examination. Although gastric PEComa is a very rare neoplasm, it should be considered in the differential diagnosis of gastric submucosal lesions.

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Unusual paediatric sigmoid perivascular epithelioid cell tumour with regional lymph node metastasis treated using gemcitabine and docetaxel: a case report and literature review
Hsiu-Chung Cheng, Chia-Yu Kuo, Ching-Wen Huang, Hsiang-Hung Shih, Chih-Hung Lin, Jaw-Yuan Wang
Journal of International Medical Research.2021; 49(9): 030006052110415. CrossRef
Gastric Perivascular Epithelioid Cell Tumor (PEComa)
Jinghong Xu, Yu Yan, Xueping Xiang, Peter Jiang, Xiangrong Hu, Wenjun Yang
American Journal of Clinical Pathology.2019; 152(2): 221. CrossRef
Robotic wedge resection of a rare gastric perivascular epithelioid cell tumor: A case report
Alessandra Marano, Francesca Maione, Yanghee Woo, Luca Pellegrino, Paolo Geretto, Diego Sasia, Mirella Fortunato, Giulio Fraternali Orcioni, Roberto Priotto, Renato Fasoli, Felice Borghi
World Journal of Clinical Cases.2019; 7(23): 4011. CrossRef

A Case of Malignant PEComa of the Uterus Associated with Intramural Leiomyoma and Endometrial Carcinoma: Yoo Jin Choi, Jin Hwa Hong, Aeree Kim, Hankyeom Kim, Hyeyoon Chang; J Pathol Transl Med. 2016;50(6):469-473. Published online July 25, 2016; DOI: https://doi.org/10.4132/jptm.2016.04.20

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Abstract PDF

Perivascular epithelioid cell tumors (PEComas) refers to a family of mesenchymal neoplasms composed of angiomyolipomas, clear cell “sugar” tumors of the lung, and lymphangioleiomyomatoses. These tumors have a distinctive and common component of perivascular epithelioid cells that show an association with blood vessel walls and immunohistochemically display myomelanocytic differentiation. The unique neoplasms have been shown to have an expanded range through a variety of case reports, including visceral, intra-abdominal, soft tissue, and bone tumors. The retroperitoneum, abdominopelvic region, and uterus have been reported to be the most common sites. Most PEComas follow a benign course. However, reports of malignant PEComas are increasing. Many papers have described uterine PEComas, but to our knowledge, there have not yet been any reports of a malignant PEComa arising concomitant with another epithelial tumor and mesenchymal tumor. We report herein the case of a 67-year-old woman who experienced a malignant uterine PEComa infiltrating a preexisting intramural leiomyoma with synchronous well differentiated endometrial carcinoma and multiple liver and lung metastases.

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Metastasis of Clear Cell Renal Cell Carcinoma to Uterine Leiomyoma: First Case Report and Review of Literature
Sarvenaz Karamooz, Paula D. Binsol, Jaya Ruth Asirvatham, Anjali Pargaonkar
International Journal of Surgical Pathology.2024;[Epub] CrossRef
Uterine collision tumor (PEComa and endometrioid carcinoma) in a tuberous sclerosis patient: a case report
Nektarios Koufopoulos, Ioannis S. Pateras, Christos Koratzanis, Alina-Roxani Gouloumis, Argyro-Ioanna Ieronimaki, Alexandros Fotiou, Ioannis G. Panayiotides, Nikolaos Vrachnis
Frontiers in Oncology.2023;[Epub] CrossRef
TFE3-associated perivascular epithelioid cell tumor with complete response to mTOR inhibitor therapy: report of first case and literature review
Roli Purwar, Kishan Soni, Mridula Shukla, Ashish Verma, Tarun Kumar, Manoj Pandey
World Journal of Surgical Oncology.2022;[Epub] CrossRef
A case of perivascular epithelioid nodules arising in an intramural leiomyoma
Yoldez Houcine, Karima Mekni, Emna Brahem, Mouna Mlika, Aida Ayadi, Chiraz Fekih, Imene Ridene, Faouzi El Mezni
Human Pathology: Case Reports.2021; 23: 200470. CrossRef
Perivascular epithelioid cell tumors (PEComa) of the female genital tract: A challenging question for gynaecologic oncologist and pathologist
Angiolo Gadducci, Gian Franco Zannoni
Gynecologic Oncology Reports.2020; 33: 100603. CrossRef
Five cases of uterine perivascular epithelioid cell tumors (PEComas) and review of literature
Weiwei Shan, Yue Shi, Qin Zhu, Bingyi Yang, Liying Xie, Bing Li, Chengcheng Ning, Qiaoying Lv, Yali Cheng, Bingying Xie, Mingzhu Bai, Yuhui Xu, Xiaojun Chen, Xuezhen Luo
Archives of Gynecology and Obstetrics.2019; 299(1): 185. CrossRef
Uterine PEComas
Jennifer A. Bennett, Ana C. Braga, Andre Pinto, Koen Van de Vijver, Kristine Cornejo, Anna Pesci, Lei Zhang, Vicente Morales-Oyarvide, Takako Kiyokawa, Gian Franco Zannoni, Joseph Carlson, Tomas Slavik, Carmen Tornos, Cristina R. Antonescu, Esther Oliva
American Journal of Surgical Pathology.2018; 42(10): 1370. CrossRef

Sclerosing Perivascular Epithelioid Cell Tumor of the Lung: A Case Report with Cytologic Findings: Ha Yeon Kim, Jin Hyuk Choi, Hye Seung Lee, Yoo Jin Choi, Aeree Kim, Han Kyeom Kim; J Pathol Transl Med. 2016;50(3):238-242. Published online April 11, 2016; DOI: https://doi.org/10.4132/jptm.2016.02.19

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Abstract PDF

Benign perivascular epithelioid cell tumor (PEComa) of the lung is a rare benign neoplasm, a sclerosing variant of which is even rarer. We present a case of 51-year-old man who was diagnosed with benign sclerosing PEComa by percutaneous fine needle aspiration cytology and biopsy. The aspirate revealed a few cell clusters composed of bland-looking polygonal or spindle cells with fine granular or clear cytoplasm. Occasional fine vessel-like structures with surrounding hyalinized materials were seen. The patient later underwent wedge resection of the lung. The histopathological study of the resected specimen revealed sheets of polygonal cells with clear vacuolated cytoplasm, variably sized thin blood vessels, and densely hyalinized stroma. In immunohistochemical studies, reactivity of tumor cells for human melanoma black 45 and Melan-A further supported the diagnosis of benign sclerosing PEComa. To the best of our knowledge, this is the first case of benign sclerosing PEComa described in lung.

Citations

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Cytopathology of rare gastric mesenchymal neoplasms: A series of 25 cases and review of literature
Carla Saoud, Peter B. Illei, Momin T. Siddiqui, Syed Z. Ali
Cytopathology.2023; 34(1): 15. CrossRef
Retroperitoneal Sclerosing Angiomyolipoma with Long-Term Follow up: A Case Report with Unique Clinicopathologic and Genomic Profile
Liwei Jia, Vandana Panwar, Michelle Parmley, Elena Lucas, Ivan Pedrosa, Payal Kapur
International Journal of Surgical Pathology.2022; 30(1): 86. CrossRef
Perivascular epithelioid cell tumor of the lung: A case report and literature review
Shaofu Yu, Shasha Zhai, Qian Gong, Xiaoping Hu, Wenjuan Yang, Liyu Liu, Yi Kong, Lin Wu, Xingxiang Pu
Thoracic Cancer.2022; 13(17): 2542. CrossRef
Cytopathology of extra-renal perivascular epithelioid cell tumor (PEComa): a series of 7 cases and review of the literature
Sintawat Wangsiricharoen, Tatianna C. Larman, Paul E. Wakely, Momin T. Siddiqui, Syed Z. Ali
Journal of the American Society of Cytopathology.2021; 10(2): 175. CrossRef
Clear cell sugar tumour: a rare tumour of the lung
Sarah Page, Matthew S. Yong, Alka Sinha, Pankaj Saxena
ANZ Journal of Surgery.2020;[Epub] CrossRef
Perivascular Epithelioid Cell Tumors (PEComas) of the Orbit
Panagiotis Paliogiannis, Giuseppe Palmieri, Francesco Tanda, Antonio Cossu
Journal of Pathology and Translational Medicine.2017; 51(1): 7. CrossRef

Epithelioid Trophoblastic Tumor: Clinicopathologic and Immunohistochemical Analysis of Three Cases: Woo Jung Sung, Hyeong Chan Shin, Min-Kyung Kim, Mi Jin Kim; Korean J Pathol. 2013;47(1):67-73. Published online February 25, 2013; DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.1.67

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Abstract PDF

Epithelioid trophoblastic tumor is an unusual type of trophoblastic tumor. Here we report on the clinicopathologic and immunohistochemical features of three cases of epithelioid trophoblastic tumor. All three patients were of reproductive age and presented with vaginal bleeding and mild elevation of human chorionic gonadotropin (hCG). All patients underwent a hysterectomy. The tumors consisted of epithelioid intermediate trophoblastic cells that were mononucleated and eosinophilic, or showed clear cytoplasm on microscopic examination. One case presented with a focal choriocarcinoma component. Immunohistochemically, the tumors displayed diffuse positivity for cytokeratin 18, E-cadherin, epidermal growth factor receptor, and p53 and focal positivity for p63 and hCG. However, expression of α-inhibin and placental alkaline phosphatase was almost negative. Tests for human placental lactogen and epithelial membrane antigen were also negative in all cases.

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Epithelioid Trophoblastic Tumour: A Case with Genetic Linkage to a Child Born over Seventeen Years Prior, Successfully Treated with Surgery and Pembrolizumab
David Pisani, Jean Calleja-Agius, Riccardo Di Fiore, John J. O’Leary, James P. Beirne, Sharon A. O’Toole, Ana Felix, Ian Said-Huntingford
Current Oncology.2021; 28(6): 5346. CrossRef
Epithelioid Trophoblastic Tumor
Stephanie M. McGregor, Larissa V. Furtado, Anthony G. Montag, Rebecca Brooks, Ricardo R. Lastra
International Journal of Gynecological Pathology.2020; 39(1): 8. CrossRef
Epithelioid trophoblastic tumor in a postmenopausal woman: A case report and review of the literature in the postmenopausal group
Seyran Yigit, Eylul Gun, Bulent Yilmaz, Zafer Kolsuz
Indian Journal of Pathology and Microbiology.2020; 63(5): 98. CrossRef
Double trouble: Extrauterine epithelioid trophoblastic tumor with uterine choriocarcinoma - An autopsy report
Kusum Jashnani, Alshifa Yagana, Niraj Mahajan
Indian Journal of Cancer.2020;[Epub] CrossRef
Epithelioid trophoblastic tumor coexisting with choriocarcinoma around an abdominal wall cesarean scar: a case report and review of the literature
Chunfeng Yang, Jianqi Li, Yuanyuan Zhang, Hanzhen Xiong, Xiujie Sheng
Journal of Medical Case Reports.2020;[Epub] CrossRef
Placental site trophoblastic tumor and epithelioid trophoblastic tumor: Clinical and pathological features, prognostic variables and treatment strategy
Angiolo Gadducci, Silvestro Carinelli, Maria Elena Guerrieri, Giovanni Damiano Aletti
Gynecologic Oncology.2019; 153(3): 684. CrossRef
Diagnosis and Management of Mixed Gestational Trophoblastic Neoplasia: A Study of 16 Cases and a Review of the Literature
Yujia Kong, Guangshi Tao, Liju Zong, Junjun Yang, Xirun Wan, Wenze Wang, Yang Xiang
Frontiers in Oncology.2019;[Epub] CrossRef
A Case Series of Five Patients With Pure or Mixed Gestational Epithelioid Trophoblastic Tumors and a Literature Review on Mixed Tumors
Ka Yu Tse, Keith Wan Hang Chiu, Karen Kar Loen Chan, Mandy Man Yee Chu, Siew Fei Ngu, Annie Nga Yin Cheung, Hextan Yuen Sheung Ngan, Philip Pun Ching Ip
American Journal of Clinical Pathology.2018; 150(4): 318. CrossRef
MR Imaging of Uterine Epithelioid Trophoblastic Tumor: A Case Report
Sakiko KAGEYAMA, Masafumi KANOTO, Yukio SUGAI, Takeshi SUTO, Satoru NAGASE, Mitsumasa OSAKABE, Takaaki HOSOYA
Magnetic Resonance in Medical Sciences.2016; 15(4): 411. CrossRef
Pharmacotherapy of placental site and epithelioid trophoblastic tumours
Fiona Taylor, Barry W Hancock
Expert Opinion on Orphan Drugs.2015; 3(1): 75. CrossRef
A Well-Circumscribed Border with Peripheral Doppler Signal in Sonographic Image Distinguishes Epithelioid Trophoblastic Tumor from Other Gestational Trophoblastic Neoplasms
Jiale Qin, Weiwen Ying, Xiaodong Cheng, Xiaodong Wu, Bingjian Lu, Yun Liang, Xinyu Wang, Xiaoyun Wan, Xing Xie, Weiguo Lu, Hai-Yan Lin
PLoS ONE.2014; 9(11): e112618. CrossRef

Case Reports

Pigmented Perivascular Epithelioid Cell Tumor (PEComa) of the Kidney: A Case Report and Review of the Literature: Hyeyoon Chang, Wonkyung Jung, Youngran Kang, Woon Yong Jung; Korean J Pathol. 2012;46(5):499-502. Published online October 25, 2012; DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.5.499

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Abstract PDF

Heavily pigmented perivascular epithelioid cell tumors (PEComa) are rare, only eight cases of which have been reported. Unlike typical epithelioid angiomyolipoma, most of these tumors have been encountered in female patients without tuberous sclerosis. The long-term prognosis thereof is undetermined. Cytological similarity and heavy melanin pigment make it difficult for pigmented PEComa to be differentiated from pigmented clear cell renal cell carcinoma or malignant melanoma. The immunoprofile of tumor cells, such as human melanoma black-45 expression, as well as the absence or presence of other melanocytic or epithelial markers, are helpful in determining a differential diagnosis. Here we report a case of heavily pigmented PEComa of the right kidney and review the literature describing this tumor. In this case, the immunoprofile and clinical features corresponded well to those described in the literature. Since the prognosis of such disease has not yet been established, close follow-up of this patient was recommended.

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Malignant Pigmented Epithelioid Angiomyolipoma of the Kidney in a Child with Tuberous Sclerosis Complex
Thu Dang Anh Phan, Nhi Thuy To, Diem Thi Nhu Pham
Fetal and Pediatric Pathology.2023; 42(2): 285. CrossRef
Perivascular epithelioid cell tumor (PEComa) of the cystic duct
Takeshi Okamoto, Takashi Sasaki, Yu Takahashi, Manabu Takamatsu, Hiroaki Kanda, Makiko Hiratsuka, Masato Matsuyama, Masato Ozaka, Naoki Sasahira
Clinical Journal of Gastroenterology.2023; 16(1): 87. CrossRef
PEComa of the Adrenal Gland
Craig B. Wakefield, Peter M. Sadow, Jason L. Hornick, Christopher D.M. Fletcher, Justine A. Barletta, William J. Anderson
American Journal of Surgical Pathology.2023; 47(11): 1316. CrossRef
Recurrence of Pigmented Epithelioid Angiomyolipoma of the Kidney With Xp11 Translocation: A Case Report
Mahmoud D Srour, Andrew Harris
Cureus.2023;[Epub] CrossRef
Pigmented perivascular epithelioid cell tumor (PEComa) arising from kidney
Hexi Du, Jun Zhou, Lingfan Xu, Cheng Yang, Li Zhang, Chaozhao Liang
Medicine.2016; 95(44): e5248. CrossRef
PEComas of the kidney and of the genitourinary tract
Guido Martignoni, Maurizio Pea, Claudia Zampini, Matteo Brunelli, Diego Segala, Giuseppe Zamboni, Franco Bonetti
Seminars in Diagnostic Pathology.2015; 32(2): 140. CrossRef
Pigmented Perivascular Epithelioid Cell Tumor of the Skin
Pooja Navale, Masoud Asgari, Sheng Chen
The American Journal of Dermatopathology.2015; 37(11): 866. CrossRef
Clear Cell Melanoma: A Cutaneous Clear Cell Malignancy
Maria A. Pletneva, Aleodor Andea, Nallasivam Palanisamy, Bryan L. Betz, Shannon Carskadon, Min Wang, Rajiv M. Patel, Douglas R. Fullen, Paul W. Harms
Archives of Pathology & Laboratory Medicine.2014; 138(10): 1328. CrossRef
Extrapulmonary Lymphangioleiomyoma: Clinicopathological Analysis of 4 Cases
Dae Hyun Song, In Ho Choi, Sang Yun Ha, Kang Min Han, Jae Jun Lee, Min Eui Hong, Yoon-La Choi, Kee-Taek Jang, Sang Yong Song, Chin A Yi, Joungho Han
Korean Journal of Pathology.2014; 48(3): 188. CrossRef

Primary Perivascular Epithelioid Cell Tumor (PEComa) of the Liver: A Case Report and Review of the Literature.: Ji Hyun Ahn, Bang Hur; Korean J Pathol. 2011;45:S93-S97.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.S1.S93

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Abstract PDF

Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor consisting of distinctive perivascular epithelioid cells, and is commonly detected in the uterus. The liver is an uncommon site for primary PEComa. In this study, we report a case of primary hepatic PEComa in a 36-year-old woman. Upon gross examination, the tumor was a well-defined, brownish solid mass, measuring 6.5x5.2x4.5 cm. Microscopically, the tumor consisted largely of epithelioid cells and some spindle cells with a clear to eosinophilic cytoplasm and a rich network of delicate capillaries in the stroma. With the exception of their relatively large size and microscopically sinusoidal infiltrative growth pattern, all other histopathologic features of the tumor were consistent with their being benign. The tumor cells were positive for human melanoma black-45 and smooth muscle actin, and negative for cytokeratin-cocktail and c-kit.

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Primary Liver Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review
Mindaugas Kvietkauskas, Austeja Samuolyte, Rokas Rackauskas, Raminta Luksaite-Lukste, Gintare Karaliute, Vygante Maskoliunaite, Ruta Barbora Valkiuniene, Vitalijus Sokolovas, Kestutis Strupas
Medicina.2024; 60(3): 409. CrossRef
Unresectable hepatic PEComa: a rare malignancy treated with stereotactic body radiation therapy (SBRT) followed by complete resection
Simon Kirste, Gian Kayser, Anne Zipfel, Anca-Ligia Grosu, Thomas Brunner
Radiation Oncology.2018;[Epub] CrossRef
Hepatic perivascular epithelioid cell tumor (PEComa): a case report with a review of literatures
Hyun Jin Son, Dong Wook Kang, Joo Heon Kim, Hyun Young Han, Min Koo Lee
Clinical and Molecular Hepatology.2017; 23(1): 80. CrossRef
Malignant hepatic perivascular epithelioid cell tumor (PEComa) – Case report and a brief review
Banerjee Abhirup, Kundalia Kaushal, Mehta Sanket, Nagarajan Ganesh
Journal of the Egyptian National Cancer Institute.2015; 27(4): 239. CrossRef
Hepatic falciform ligament clear cell myomelanocytic tumor: A case report and a comprehensive review of the literature on perivascular epithelioid cell tumors
Zu-Sen Wang, Lin Xu, Lin Ma, Meng-Qi Song, Li-Qun Wu, Xuan Zhou
BMC Cancer.2015;[Epub] CrossRef
Improving recognition of hepatic perivascular epithelioid cell tumor: Case report and literature review
Toshiya Maebayashi
World Journal of Gastroenterology.2015; 21(17): 5432. CrossRef
Primary perivascular epithelioid cell tumor of the liver: new case report and literature review
Hassania Ameurtesse, Laïla Chbani, Amal Bennani, Imane Toughrai, Nouhad Beggui, Imane Kamaoui, Hinde Elfatemi, Taoufik Harmouch, Afaf Amarti
Diagnostic Pathology.2014;[Epub] CrossRef
Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features—analysis of 7 cases and review of the literature
Yan Tan, En-hua Xiao
Abdominal Radiology.2012; 37(5): 781. CrossRef

Fine Needle Aspiration Cytology of Pulmonary Epithelioid Hemangioendothelioma with Prominent Hyaline Degeneration: A Case Report.: Kang Min Han, Dong Hoon Kim, Na Hye Myong; Korean J Pathol. 2010;44(5):554-557.; DOI: https://doi.org/10.4132/KoreanJPathol.2010.44.5.554

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Abstract PDF

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor of low to intermediate malignant potential, and PEH can mimic other more common tumor entities pathologically as well as clinically. Compared to its well-recognized histological features, its cytological findings have been reported rarely to be plasmacytoid or epithelioid cells with abundant dense or finely granular cytoplasm, cytoplasmic vacuoles, round nuclei and prominent nucleoli.We report here on the fine needle aspiration cytologic findings of a 38-year-old woman with EH of the lung, that showed in addition to its classical cytomorphology, a somewhat peculiar cytologic finding such as big twig-like rosettoid structures with prominent hyalinized stroma. This tumor was histologically and immunohistochemically proven to be PEH by primary antibodies for CD31, CD34 and vimentin. We emphasize that the accuracy of making a cytologic diagnosis of this rare tumor can be increased by recognizing the peculiar cytologic finding that we report on here.

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Pulmonary epithelioid hemangioendothelioma misconceived as pulmonary metastasis of other malignancies
Gi Tark Noh, Kyoung Ju Lee, Hee Jung Sohn, Kyung Han Lee, Won Seok Heo, Byung Sung Koh, Un Mi Han, Young A Bae
Yeungnam University Journal of Medicine.2016; 33(1): 72. CrossRef
Fine needle aspiration cytology of epithelioid hemangioendothelioma of soft tissue
Shelly Sehgal, Reena Agarwal, Sarika Verma, Ajay Kumar Verma, Sompal Singh
Diagnostic Cytopathology.2013; 41(2): 179. CrossRef

Epithelioid Hemangioendothelioma of Liver: A case report.: Yoo Jin Kim, Jae Hwa Lee, Bang Hur, Man Ha Hur; Korean J Pathol. 1995;29(3):378-384.

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Abstract PDF: Epithelioid hemangioendothelioma of liver is a very rare tumor of vascular origin, the most peculiar feature of which is that it is composed of endothelial cells closely resembling epithelial cells. We present a primary epithelioid hemangioendothelioma of liver in a 40-year-old male. This tumor was composed of an ill-defined yellowish white, 7 x 4cm sized, firm, solid mass and small satellite nodules in the right lobe of liver. Microscopically, two types of tumor cells-dendritic and epithelioid-were identified. The neoplastic cells infiltrated into sinusoids and intrahepatic veins. The background of tumor showed marked sclerotic change and focal proliferation of bile ductules. Confirmation of the endothelial origin of these cells was provided by positive immunoperoxidase staining for factor VIII-related antigen, and by electron mi-croscopic demonstration of Weibel-Palade body. This is the first case of epithelioid heman- gioendothelioma of liver documented in Korea. We report this case in view of its scarcity and distinctive morphologic features that allow differentiation from sclerosing carcinoma and angiosarcoma.

Coexisting Adenocarcinoma and Epithelioid Leiomyosarcoma of Stomach: A case report.: Jung Youn Kim, Jae Soo Koh, Kyung Ja Cho, Ja June Jang; Korean J Pathol. 1995;29(4):521-523.

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Abstract PDF: We report a case of malignant tumor of the stomach showing both carcinoma and sarcoma components in a 47 year-old woman. The resected stomach revealed two tumors which were in different location. The one mass was a 2 x I cm, poorly demarcated tumor at the body, along the lesser curvature and the other was a 6 x 7 cm, well demarcated polypoid tumor with a central depression at cardia of the posterior stomach wall. The tumor at the body was a poorly differentiated adenocarcinoma which showed strong reactivity for CEA. The tumor at the cardia was composed of epithelioid or spindle shaped cells with abundant eosinophilic cytoplasm and revealed 5-6 mitoses per ten high power field. The tumor at the cardia was diagnosed as epithelioid leiomyosarcoma and showed reactivity for vimentin but none for actin, desmin,keratin, and CEA. This is different from the usual carcinosarcoma.

Syphilitic Granulomatous Pancreatitis: A case report.: Seong Eun Yang, Yoon Ju Kim, Sung Suk Paeng, Duck Hwan Kim, Hee Jin Chang, Jung Il Suh; Korean J Pathol. 1996;30(8):721-725.

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Abstract PDF: Syphilitic granulomatous pancreatitis is an extremely rare condition,and can occur in the generalized acquired syphilitic patient in tertiary or secondary phase. The most serious problem with granulomatous pancreatic lesion is clinical or radiological misdiagnosis as cancer. We experienced a case of syphilitic granulomatous pancreatitis arising in 54 year old female patient. She was treated for syphilis 20years ago. But she and her husband are still strong positive to VDRL and TPHA. On abdominal computed tomography and endoscopic pancreatico- duodenography, there was an obstructive mass of low density in the distal common bile duct or pancreatic head. Under the preoperative diagnosis of pancreatic head carcinoma, Whipple's operation was done. On gross examination, the pancreas was fibrotic, and the common bile duct was well preserved without tumor mass. Microscopically, numerous intralobular noncaseating epithelioid cell granulomas with multinucleated giant cells are identified. They surround thick-walled, small to medium sized arteries and involve vascular wall with luminal narrowing or obliteration, which are characteristic findings of the syphilitic granuloma. The remaining parenchyme shows fibrosis, acinar atrophy or destruction with dense infiltration of lymphohistiocytes, plasma cells with granuloma formation. Although the Warthin-Starry stain reveals no spirochetes, the serologic result and pathologic findings are compatible with syphilitic granulomatous pancreatitis.

Epithelioid Leiomyosarcoma of Skin: A case report.: Han Seong Kim, Nam Bok Cho, Hyun Soon Lee, Kye Yong Song; Korean J Pathol. 1998;32(9):700-703.

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Abstract: This report concerns a rare malignant smooth muscle neoplasm of the skin and the subcutaneous tissue, which was examined immunohistochemically and ultrastructurally. It occured in a 48-year-old female patient who had suffered from painful nodules on the left shoulder. The nodules were removed and diagnosed as a benign fibrohistiocytic tumor. One year after local excision, multiple nodules were detected by physical examination at the previous operation site. Grossly, thirteen well-defined small nodules had spread in the dermis and the subcutaneous tissue, of which nine were located from lower dermis to the subcutaneous tissue and four were in the subcutaneous tissue. Histologically, each nodule consisted mainly of epithelioid tumor cells having eosinophilic or clear plump cytoplasm and round to oval nuclei with small nucleoli, and some peripheral spindle tumor cells. The tumor cells were negative for PAS reaction with and without diastase digestion or alcian blue. Immunohistochemically, tumor cells were positive for smooth muscle actin and vimentin. Ultrastructurally, the tumor cells showed actin filaments and dense bodies in cytoplasm. This case was diagnosed as an epithelioid leiomyosarcoma of the skin and the subcutaneous tissue with local metastasis one year after local excision.

Epithelioid Leiomyosarcoma of Retroperitoneum: A case report.: Myeng Sun Park, Ji Young Seo, Hae Jin Jeong, Bang Hur, Man Ha Hur; Korean J Pathol. 1999;33(2):141-144.

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Abstract: Epithelioid smooth muscle tumor is relatively rare and potentially malignant, especially in retroperitoneum. Distinct criteria for malignancy still have not been clarified in this epithelioid variant arising in retroperitoneum. We report a deceptively benign-appearing epithelioid leiomyosarcoma in a 50-year-old female. She was admitted with abdominal discomfort and dysuria. Abdominal CT showed a well-demarcated, 10 10 cm sized, solid mass in retroperitoneum. Concomitant metastatic lesions were noted in right lung field. Surgical excision of retroperitoneal mass and right lung lobectomy were performed. The retroperitoneal mass showed yellowish-tan, well-delineated and lobulated appearance. Histologically, this tumor was composed of predominantly epithelioid, round to oval cells with distinct clear cytoplasm and slightly atypical nuclei. Mitosis was rare (0~1/50 HPF). Lung lesions were morphologically similar to that of retroperitoneum.

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