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[A large para-renal PEComa].

Virginie Audard1, Maryline Dorel-Le Théo, Marie-Dominique Trincard

  • 1Service d'Anatomie et Cytologie Pathologique, Hôpital Cochin, 27 rue du Faubourg Saint Jacques, 75769 Paris Cedex 14.

Annales De Pathologie
|October 14, 2004
PubMed
Summary
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Epithelioid AML (PEComa) can be challenging to diagnose and may recur. High mitotic count and tumor size may indicate malignancy in these rare tumors.

Area of Science:

  • Oncology
  • Pathology

Background:

  • Perivascular Epithelioid Cell Tumors (PEComas) are rare mesenchymal neoplasms.
  • Epithelioid Angiomyolipoma (AML) is a subtype of PEComa.
  • Diagnosis can be challenging due to overlapping features with other tumors.

Observation:

  • A 45-year-old male presented with a large pararenal mass.
  • Histopathology revealed epithelioid and spindle cells with atypia and high mitotic count.
  • Immunohistochemistry was positive for HMB45 and actin, negative for epithelial markers and PS100.

Findings:

  • The diagnosis of epithelioid AML (PEComa) was confirmed.
  • Recurrence occurred two years later with a large abdominal mass.
  • The recurrent tumor exhibited similar morphological and immunohistochemical features.

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Implications:

  • Epithelioid AMLs can have an unfavorable prognosis with metastatic potential.
  • Malignancy criteria for PEComas are not well-defined.
  • Mitotic count and tumor size may be important indicators of malignancy in epithelioid AML.