Primary neuroectodermal tumor of penis (PNET)--a case report.

Primary neuroectodermal tumor (PNET) is a rare entity with high malignant potential. It usually affects the skeletal system and primary extraskeletal involvement is uncommon. Because of rarity of tumor in the penis, only a few cases have been reported. A 17-year-old male adolescent presented with painless penile swelling of 4 weeks' duration. Clinical examination revealed multiple, 1 cm hard nodules palpable on penile shaft. Open incisional biopsy of the penile nodule revealed an undifferentiated malignant round cell tumor and on immunohistochemistry a diagnosis of PNET was made. Metastatic workup was negative. Total penectomy and perineal urethrostomy was performed, and subsequently adjuvant chemotherapy was given.