Desmoplastic Small Round Cell Tumor
Image Description
Image courtesy of Dr. Jean-Christophe Fournet, Paris, France; humpath.com; Used with permission.
About the Disease
Malignant tumors of testicular adnexa are quite rare and include genitourinary sarcomas, mesotheliomas, carcinoma of the epididymis, and metastases. Genitourinary sarcomas in adults usually involve spermatic cord, epididymis and surrounding soft tissues. The most common genitourinary sarcoma in adults is well-differentiated liposarcoma, followed by leiomyosarcoma, undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma), and embryonal rhabdomyosarcoma. Embryonal rhabdomyosarcoma is the most common histologic subtype in children and men below 30 yrs. Most of these tumors are bulky and present as painless, palpable mass. Besides embryonal rhabdomyosarcoma, another malignant tumor that may involve the paratesticular region in children is desmoplastic small cell tumor. Mesothelioma may arise from the tunica vaginalis and present as a painless scrotal mass along with a hydrocele. It may be associated with asbestos exposure.Paratesticular sarcomas are managed with wide excision of the spermatic cord and testis with high ligation via an inguinal approach. Incompletely resected tumors are treated by repeat wide local excision and/or post-operative radiation. The long-term survival of men with paratesticular sarcomas is around 50%.