PEComa

PEComa (Perivascular Epithelioid Cell tumors) are a group of rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. They are characterized by the co-expression of melanocytic and smooth muscle markers.

Classification

PEComas are classified into the following categories:

• Angiomyolipoma
• Clear cell "sugar" tumor of the lung
• Lymphangioleiomyomatosis
• Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres
• Malignant PEComa
• Other/unclassified PEComas

Pathology

PEComas are characterized by the presence of perivascular epithelioid cells. These cells have a characteristic round to oval shape, clear to eosinophilic cytoplasm, and centrally located nuclei. They are typically arranged around blood vessels.

Diagnosis

The diagnosis of PEComa is based on the histological and immunohistochemical features of the tumor. The tumor cells are typically positive for melanocytic markers (such as HMB-45 and Melan-A) and smooth muscle markers (such as desmin and smooth muscle actin).

Treatment

The treatment of PEComa depends on the type and location of the tumor. Surgical resection is the mainstay of treatment for localized tumors. For advanced or metastatic tumors, systemic therapy may be considered.

Prognosis

The prognosis of PEComa depends on various factors such as the size and location of the tumor, the presence of metastasis, and the patient's overall health status.

See also

• Tumor
• Cancer
• Pathology
• Oncology

PEComa Resources
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