Anatomic Pathology: Genitourinary Pathology

• Pure testicular primitive neuroectodermal tumor (PNET) is a very infrequent finding, and is considered a monodermal teratoma. It is more commonly seen in the setting of a germ cell tumor as a secondary malignant transformation, usually associated with a teratomatous component, although even in this setting, it is also quite rare.

• Histologically, the same spectrum of patterns seen in peripheral PNET/Ewing sarcoma of other sites can be seen; however, gonadal PNET tend to be more often similar to central nervous system tumors of the embryonic type, seen in the pediatric population. Immunohistochemically, FLI-1 and CD99 positivity are frequently seen, with variable expression of CD57, synaptophysin, neuron-specific enolase (NSE) and chromogranin A.

• Gonadal PNET lack chromosome 22 rearrangements seen in peripheral Ewing/PNET.

• The presence of a secondary malignant transformation arising from a testicular germ cell tumor, such as PNET or rhabdomyosarcoma, generally depicts a worse prognosis. Patients with PNET confined to the primary testicular germ cell tumor have a better prognosis than patients with PNET arising in metastatic sites, which tend to have a dismal prognosis.

 
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