Micronodular PEComa of the appendix: a case report - Takeaways - MDSpire

Micronodular PEComa of the appendix: a case report

  • By

  • Li-Jing Jiang

  • Chao-Qun Wang

  • Zheng-Guo Xu

  • May 22, 2026

  • 0 min

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  • 1

    Micronodular perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm of the appendix, recently reclassified within the PEComa family.

  • 2

    A 23-year-old man with recurrent abdominal pain underwent laparoscopic appendectomy, revealing multiple micronodular nests of epithelioid cells.

  • 3

    Histological examination showed abundant eosinophilic granular cytoplasm and small nuclei, with immunohistochemical positivity for SMA, Desmin, and HMB-45.

  • 4

    The Ki-67 proliferation index was less than 1%, indicating low mitotic activity and supporting the diagnosis of micronodular PEComa.

  • 5

    At 8 months post-surgery, the patient reported significant improvement in symptoms, with no clinical evidence of recurrence.

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