Primary Multifocal Hepatic PEComa: A Rarely Encountered Diagnostic Dilemma

  • Tripti Nakra
  • Prasenjit Das
  • Brijnandan Gupta
  • Rajni Yadav
  • Anand N. Singh
  • Nihar R. Dash
  • K. S. Madhusudhan
  • Prashant RamtekeEmail author
Case Report


The origin of perivascular epithelioid cell neoplasm (PEComa) family of tumors was first proposed by Bonetti et al. [1] in 1992, and in 2002 World Health Organization (WHO) defined PEComa as “mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells” [2]. 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 [3]. 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 [4]. Although PEComas show a wide anatomical distribution, most commonly they arise in the retroperitoneum, abdominopelvic region, uterus, and gastrointestinal tract. Hepatic PEComas are rare [4] with only a few cases of...


Author Contribution

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.

Funding Information


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest


  1. 1.
    Bonetti F, Pea M, Martignoni G, et al. PEC and sugar. Am J Surg Pathol. 1992;16:307–8.CrossRefGoogle Scholar
  2. 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. 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
  4. 4.
    Son HJ, Kang DW, Kim JH, Han HY, Lee MK. Hepatic perivascular epithelioid cell tumor (PEComa): a case report with a review of literatures. Clin Mol Hepatol. 2017;23(1):80–6.CrossRefGoogle Scholar
  5. 5.
    Lan YZ, Hua XE. Hepatic multiple perivascular epithelioid cell neoplasm: a case report and literature review. Mol Clin Oncol. 2016;4(4):619–21.CrossRefGoogle Scholar
  6. 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
  7. 7.
    Folpe AL, Kwiatkowski DJ. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 2010;41:1–15.CrossRefGoogle Scholar
  8. 8.
    Flemming P, Lehmann U, Becker T, Klempnauer J, Kreipe H. Common and epithelioid variants of hepatic angiomyolipoma exhibit clonal growth and share a distinctive immunophenotype. Hepatology. 2000;32:213–7.CrossRefGoogle Scholar
  9. 9.
    Mai KT, Yazdi HM, Perkins DG, Thijssen A. Fine needle aspiration biopsy of epithelioid angiomyolipoma. A case report. Acta Cytol. 2001;45:233–6.CrossRefGoogle Scholar
  10. 10.
    Hino A, Hirokawa M, Takamura K, Sano T. Imprint cytology of epithelioid angiomyolipoma in a patient with tuberous sclerosis. A case report. Acta Cytol. 2002;46:545–9.CrossRefGoogle Scholar
  11. 11.
    Alatassi H, Sahoo S. Epithelioid angiomyolipoma of the liver with striking giant cell component: fine-needle aspiration biopsy findings of a rare neoplasm. Diagn Cytopathol. 2009;37:192–4.CrossRefGoogle Scholar
  12. 12.
    Xu PJ, Shan Y, Yan FH, Ji Y, Ding Y, Zhou ML. Epithelioid angiomyolipoma of the liver: cross-sectional imaging findings of 10 immunohistochemically-verified cases. World J Gastroenterol. 2009;15:4576–81.CrossRefGoogle Scholar
  13. 13.
    Xie L, Jessurun J, Manivel JC, Pambuccian SE. Hepatic epithelioid angiomyolipoma with trabecular growth pattern: a mimic of hepatocellular carcinoma on fine needle aspiration cytology. Diagn Cytopathol. 2012;40:639–50.CrossRefGoogle Scholar
  14. 14.
    Tan Y, Xiao EH. Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features-analysis of 7 cases and review of the literature. Abdom Imaging. 2012;37(5):781–7.CrossRefGoogle Scholar
  15. 15.
    Maebayashi T, Abe K, Aizawa T, Sakaguchi M, Ishibashi N, Abe O, et al. Improving recognition of hepatic perivascular epithelioid cell tumor: case report and literature review. World J Gastroenterol. 2015;21(17):5432–41.CrossRefGoogle Scholar
  16. 16.
    Liao W, Ding ZY, Zhang B, et al. Primary functioning hepatic paraganglioma mimicking hepatocellular carcinoma: a case report and literature review. Medicine (Baltimore). 2018;97(17).CrossRefGoogle Scholar
  17. 17.
    Cassol C, Mete O. Endocrine manifestations of Von Hippel–Lindau disease. Arch Pathol Lab Med. 2015;139(2):263–8.CrossRefGoogle Scholar
  18. 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
  19. 19.
    Zhou Y, Chen F, Jiang W, Meng Q, Wang F. Hepatic epithelioid angiomyolipoma with an unusual pathologic appearance: expanding the morphologic spectrum. Int J Clin Exp Pathol. 2014;7(9):6364–9.PubMedPubMedCentralGoogle Scholar
  20. 20.
    Parfitt JR, Bella AJ, Izawa JI, et al. Malignant neoplasm of perivascular epithelioid cells of the liver. Arch Pathol Lab Med. 2006;130:1219–22.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2020

Authors and Affiliations

  1. 1.Department of PathologyAll India Institute of Medical SciencesNew DelhiIndia
  2. 2.Department of Gastrointestinal SurgeryAll India Institute of Medical SciencesNew DelhiIndia
  3. 3.Department of Radio DiagnosisAll India Institute of Medical SciencesNew DelhiIndia

Personalised recommendations