Objective: To investigate the clinical and pathological characteristics, treatment and prognosis of peripheral primitive neuroectodermal tumor (PNET) of the urinary tract and reproductive system.

Methods: The clinical data and pathological characteristics of a PNET patient was analyzed and relevant literature reviewed.

Results: The diagnosis was established by pathological and immunohistochemical method. The patient underwent radical surgery, followed by chemotherapy.

Conclusion: Pathology and immunohistochemistry help the diagnosis of PNET. For the treatment of the tumors in the early stage, surgery is the best choice, and for that in the late stage, it can be followed by chemotherapy. The PNET of the penis is a rare disease and evidence still lacks for the evaluation of its prognosis.

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