Purpose of review: The presence of vascular solid tumors within the testicle is considered to be malignant until proven otherwise. However, it is prudent for clinicians to be aware of rare benign and malignant intratesticular lesions as management can differ from the established treatment algorithms for germ-cell tumors.
Recent findings: Utilizing certain histopathologic findings can assist with the diagnosis of rare testis tumors. Often times the tumor subtypes are an important consideration in the grading and classification of the disease, which drives management. The multidisciplinary management of rare malignant testis tumors at an experienced center seems to provide optimal patient outcomes. Regardless of the primary treatment, prolonged follow-up for sex cord stromal tumors and other rare testis malignancies is advocated due to the delayed metastatic potential.
Summary: The clinical presentation of rare benign and malignant testis tumors is often similar to that of germ-cell tumors. Likewise, imaging characteristics are also often indistinguishable. However, the management of these rare tumors is often different from the well established treatment algorithms of germ-cell tumors. To that end, it is important for the practicing urologist to be familiar with the current principles of these tumor characteristics and the management.