Durable Disease Control With Nivolumab in Malignant Epithelioid Angiomyolipoma: A Case Report

Epithelioid angiomyolipoma is a subtype of perivascular epithelioid cell tumors that may exhibit malignant behavior. While mammalian target of rapamycin inhibitors are commonly used for systemic treatment, the efficacy of immune checkpoint inhibitors remains unclear.

A 58-year-old man was diagnosed with epithelioid angiomyolipoma after partial nephrectomy of a right renal mass. The tumor recurred locally, and multiple lung metastases developed despite surgical resection. Nivolumab was initiated after disease progression on everolimus, resulting in durable disease control for over 3 years. Immunohistochemistry revealed high PD-L1 expression and CD8+ T-cell infiltration within the primary tumor.

Immune checkpoint inhibitors may be effective treatment options for malignant epithelioid angiomyolipoma with PD-L1 expression and CD8+ T-cell infiltration.