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Sex Cord-Stromal Tumors of the Testis

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Rare Genitourinary Tumors

Abstract

Sex cord-stromal tumors (SCST) are very rare, representing approximately 4 % of all testis tumors and about 8 % of testis tumors in prepubertal males. Most resemble or recapitulate normal testicular components such as Leydig cells, Sertoli cells, and nonspecific stromal cells. SCST usually present as a painless testicular mass, but some may be associated with endocrine manifestations such as isosexual precocity, or gynecomastia, or erectile dysfunction. Most SCST are sporadic, but some show particular clinical associations with Peutz-Jeghers syndrome, Carney complex, and androgen insensitivity syndrome. In certain cases, the diagnosis of SCST may be the presenting feature for one of these syndromes.

Most are biologically benign, but about 10 % may show malignant behavior. In general, the definitive criterion for malignancy is the finding of metastasis, but certain pathologic features may connote a greater risk for subsequent malignant behavior. The details vary between tumor types, but malignant tumors usually include several of the following features: large tumor size, the presence of tumor necrosis, lymph-vascular space invasion, nuclear atypia, and a high mitotic rate.

The mainstay of treatment for SCST is orchiectomy with testis-sparing surgery a possibility for some tumors. Involvement of the retroperitoneal lymph nodes generally leads to retroperitoneal lymph node dissection. The finding of malignant pathologic features in the primary tumor necessitates close follow-up and may lead to a retroperitoneal lymph node dissection for possible occult disease. Malignant SCST generally are not sensitive to chemotherapy or radiotherapy.

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Barry, M., Rao, A., Lauer, R. (2016). Sex Cord-Stromal Tumors of the Testis. In: Pagliaro, L. (eds) Rare Genitourinary Tumors. Springer, Cham. https://doi.org/10.1007/978-3-319-30046-7_16

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