Skip to main content
Log in

Primary Multifocal Hepatic PEComa: A Rarely Encountered Diagnostic Dilemma

  • Case Report
  • Published:
Journal of Gastrointestinal Cancer Aims and scope Submit manuscript

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4

References

  1. Bonetti F, Pea M, Martignoni G, et al. PEC and sugar. Am J Surg Pathol. 1992;16:307–8.

    Article  CAS  Google Scholar 

  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.

  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. 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.

    Article  Google Scholar 

  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.

    Article  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.

  7. Folpe AL, Kwiatkowski DJ. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 2010;41:1–15.

    Article  CAS  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  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.

    Article  CAS  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

    Article  Google Scholar 

  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.

  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.

    Article  Google Scholar 

  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).

  17. Cassol C, Mete O. Endocrine manifestations of Von Hippel–Lindau disease. Arch Pathol Lab Med. 2015;139(2):263–8.

    Article  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.

  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.

    PubMed  PubMed Central  Google Scholar 

  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.

    PubMed  Google Scholar 

Download references

Funding

Nil

Author information

Authors and Affiliations

Authors

Contributions

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.

Corresponding author

Correspondence to Prashant Ramteke.

Ethics declarations

Conflict of Interest

The authors declare that they have no conflict of interest

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Nakra, T., Das, P., Gupta, B. et al. Primary Multifocal Hepatic PEComa: A Rarely Encountered Diagnostic Dilemma. J Gastrointest Canc 51, 667–672 (2020). https://doi.org/10.1007/s12029-019-00352-4

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12029-019-00352-4

Navigation