Testicular Signet Ring Stromal Tumor

**Semantic type:** Neoplastic Process

**Definition:** An extremely rare tumor that arises from the testis. It is characterized by the proliferation of epithelioid cells with signet ring appearance separated by fibrous tissue into trabeculae and nests. The histologic features are bland and there is no mucin production. Mutations of CTNNB1 gene have been identified in the reported cases. It is considered a special type of sex cord-stromal tumor. All reported cases had a benign clinical course without metastases.

**Synonyms:** - PSRSTT - Primary Signet Ring Stromal Tumor of the Testis - Testicular Signet-Ring Stromal Tumor