Primary Multifocal Hepatic PEComa: A Rarely Encountered Diagnostic Dilemma
- 8 Downloads
The origin of perivascular epithelioid cell neoplasm (PEComa) family of tumors was first proposed by Bonetti et al.  in 1992, and in 2002 World Health Organization (WHO) defined PEComa as “mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells” . In 2013, the WHO redefined them as mesenchymal tumors composed of distinctive cells that show a focal association with blood vessel walls and co-express melanocytic and smooth muscle markers . This group of mesenchymal neoplasm includes angiomyolipoma (AML), clear cell sugar tumor (CCST) of the lung, lymphangioleiomyomatosis (LAM), and a variety of intraabdominal, visceral, and soft tissue tumors with similar immunophenotypical features . Although PEComas show a wide anatomical distribution, most commonly they arise in the retroperitoneum, abdominopelvic region, uterus, and gastrointestinal tract. Hepatic PEComas are rare  with only a few cases of...
Conceptualization: Prashant Ramteke and Prasenjit Das. Data curation and investigation: Brijnandan Gupta, Anand N. Singh, Nihar R Dash, and Madhusudhan KS. Literature review: Tripti Nakra, Prasenjit Das, and Rajni Yadav. Writing-original draft preparation: Tripti Nakra. Writing-review and editing: Prashant Ramteke and Prasenjit Das. Supervision: Prashant Ramteke, Prasenjit Das, and Rajni Yadav.
Compliance with Ethical Standards
Conflict of Interest
The authors declare that they have no conflict of interest
- 2.Fletcher CDM, Unni KK, Mertens F. World Health Organization classification of tumours. In: Mertens F, ed. Pathology and genetics of soft tissue and bone. Lyon: IARC Press;2002:221–2.Google Scholar
- 3.Fletcher CDM, Bridge JA, Hogendoorn PCW, et al. World Health Organization classification of tumours of soft tissue and bone. 4th ed, vol. 5. Lyon: IARC Press; 2013. p. 230–1.Google Scholar
- 6.Khan HM, Katz SC, Libbey NP, et al. Hepatic PEComa: a potential pitfall in the evaluation of hepatic neoplasms. BMJ Case Rep. 2014.Google Scholar
- 18.Miettinen M, Sciot R, Tsui WM. Gastrointestinal clear cell sarcoma/malignant gastrointestinal neuroectodermal tumor. In: Lokuhetty D, White VA, Watanabe R, Cree IA, editors. WHO classification of Tumours editorial board. Digestive system tumors. 5th ed. IARC: Lyon; 2019. p. 494–6.Google Scholar