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  1. Home
  3. Hepatic And Perihepatic Pecomas: A Study Describing A Series Of Five Rare Cases.
  1. Home
  3. Hepatic And Perihepatic Pecomas: A Study Describing A Series Of Five Rare Cases.

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Hepatic and perihepatic PEComas: A study describing a series of five rare cases.

P Vijayanirmala1, Rajni Yadav1, Surbhi Goyal2

  • 1Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.

Indian Journal of Pathology & Microbiology
|March 1, 2024

View abstract on PubMed

Summary
This summary is machine-generated.

Accurate diagnosis of rare hepatic perivascular epithelioid cell tumors (PEComas) is crucial. This study reviewed five cases, emphasizing histological evaluation to prevent misdiagnosis and aggressive treatment for these mesenchymal neoplasms.

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Area of Science:

  • Oncology
  • Pathology
  • Rare Cancers

Background:

  • Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms with dual melanocytic and muscular differentiation.
  • Hepatic PEComas are exceptionally rare, posing diagnostic challenges and having an unclear clinical behavior.

Purpose of the Study:

  • To report and analyze five cases of hepatic and perihepatic PEComas.
  • To highlight the importance of accurate histological diagnosis for appropriate patient management.

Main Methods:

  • Retrospective review of five hepatic and perihepatic PEComa cases over five years.
  • Detailed histological evaluation and comprehensive immunohistochemical staining.
  • Fluorescence in-situ hybridization (FISH) analysis for TFE3 gene rearrangement.

Main Results:

  • All five patients were women, with an average age of 44.
  • Lesions were located in the right hepatic lobe (3), left hepatic lobe (1), and gastrohepatic ligament (1).
  • Preoperative diagnoses varied, including hepatocellular carcinoma (HCC) and metastatic tumors, underscoring diagnostic difficulty.

Conclusions:

  • Accurate histological diagnosis of hepatic PEComas is vital to avoid overtreatment.
  • Distinguishing PEComas from primary liver cancers or metastases is critical for patient management.