Renal angiomyoadenomatous tumor (RAT): a rare distinct entity with diagnostic challenges-a case report

Renal angiomyoadenomatous tumor (RAT) is a recently described rare renal neoplasm with variations in the presentation, gross, and microscopic findings, and having a benign course and good prognosis. It is characterized microscopically by the admixture of three components-epithelial cells arranged in tubules and nests, angiomyomatous stroma, and capillary-sized interconnecting vascular channels in close association with the epithelial cell clusters. Microscopically, these tumors can be confused with clear cell carcinoma, papillary carcinoma, mixed epithelial and stromal tumors, and angiomyolipoma. RAT differs from conventional clear cell carcinomas, which can rarely be associated with an identical leiomyomatosis stroma occasionally forming abortive vascular structures. RAT is a distinct morphologic entity, being different morphologically, immunohistochemically, and genetically from all renal tumors including conventional clear cell carcinoma and mixed epithelial and stromal tumor of the kidney.

Here, we report a case of a 21-year-old man with renal angiomyoadenomatous tumor, a rare neoplasm with only a few previous cases reported in the literature. Unlike our case, most tumors have been identified in middle-aged males; they present as well-circumscribed, encapsulated tan-brown masses with variably prominent cystic areas.

Diagnosis of RAT is challenging because of the rarity of the disease and common presenting symptoms to other renal pathology and is supplemented with histopathology and immunohistochemistry. A multidisciplinary team approach for diagnosis and management along with long-term follow-up are warranted.