Pulmonary Perivascular Epithelioid Cell Tumor (Pecoma). Case Report and Review of The Topic

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Background: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms with dual melanocytic and myogenic differentiation. Pulmonary PEComas, also called clear cell sugar tumors, are extremely uncommon and usually appear as solitary nodules. Their radiological features often mimic malignancy, creating a diagnostic challenge. Case presentation: We report a 55-year-old female with an incidental solitary pulmonary nodule identified during preoperative evaluation for cataract surgery. Her medical history included hypertension, type 2 diabetes mellitus, and smoking. Biopsy confirmed pulmonary PEComa, with immunohistochemical positivity for Melan-A, HMB- 45, and Actin. The patient underwent right posterolateral thoracotomy with mediolateral lobectomy. Postoperative recovery was favorable. Discussion: Pulmonary PEComas exhibit variable clinical behavior, ranging from benign to malignant. Accurate diagnosis relies on histological and immunohistochemical markers to distinguish them from morphologically similar tumors, such as metastatic melanoma or renal cell carcinoma. Complete surgical excision remains the standard treatment, as no established adjuvant therapies currently exist. Long-term surveillance is essential, particularly in cases showing malignant features. Novel targeted therapies, including mTOR inhibitors, are being investigated for recurrent or aggressive forms. Conclusion: This case emphasizes the need for a multidisciplinary approach in managing pulmonary PEComas. Although typically benign, careful histopathological evaluation and ongoing follow-up are crucial due to their malignant potential. Additional studies are necessary to clarify prognostic factors and refine therapeutic strategies.

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