Micronodular PEComa of the appendix: a case report
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By
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Li-Jing Jiang
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Chao-Qun Wang
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Zheng-Guo Xu
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May 22, 2026
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Clinical Scorecard: Micronodular Perivascular Epithelioid Cell Tumor of the Appendix: A Case Study
At a Glance
| Category | Detail |
|---|
| Condition | Micronodular Perivascular Epithelioid Cell Tumor (PEComa) of the Appendix |
| Key Mechanisms | Rare mesenchymal neoplasm with epithelioid-to-spindle cells displaying perivascular distribution and co-expressing melanocytic and myogenic markers. |
| Target Population | Adult patients, specifically a case reported in a 23-year-old male. |
| Care Setting | Hospital setting, specifically during laparoscopic appendectomy. |
Key Highlights
- Extremely rare subtype of PEComa with limited documented cases.
- Histological features include micronodular nests of epithelioid cells with eosinophilic granular cytoplasm.
- Immunohistochemical analysis shows positivity for SMA, Desmin, and HMB-45.
- Ki-67 proliferation index is less than 1%, indicating low mitotic activity.
- Patient showed marked improvement post-surgery with no recurrence at follow-up.
Guideline-Based Recommendations
Diagnosis
- Histological examination revealing epithelioid cell nests.
- Immunohistochemical analysis to confirm diagnosis.
Management
- Laparoscopic appendectomy as the primary treatment.
Monitoring & Follow-up
- Follow-up to assess for recurrence post-surgery.
Risks
- Potential for misdiagnosis due to subtle histologic features.
Patient & Prescribing Data
23-year-old male with recurrent right lower quadrant pain.
Surgical intervention led to significant symptom relief.
Clinical Best Practices
- Consider PEComa in differential diagnosis for appendiceal tumors.
- Utilize immunohistochemical markers for accurate diagnosis.
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