Malignant peripheral nerve sheath tumors (MPNSTs) are soft tissue sarcomas that arise from peripheral nerve fibers and primarily occur in the setting of neurofibromatosis (NF1). MPNST arising from the penis is very rare and may require mutilating surgery to achieve surgical cure. We previously reported a case of MPNST involving the penis in a 14-month-old boy treated with neoadjuvant chemotherapy, total penectomy, and adjuvant radiation. Here we report intermediate follow-up of the same patient, describe his subsequent genitourinary reconstruction, and discuss management dilemmas that arise following treatment of penile MPNST.
Urology. 2019 Oct 02 [Epub ahead of print]
Hannah Agard, Neel Parekh, Curtis Clark, Eric Massanyi, Ananth Murthy, Daniel McMahon
Department of Urology, Cleveland Clinic Akron General, Akron, OH. Electronic address: ., Department of Urology, Cleveland Clinic Akron General, Akron, OH., Pediatric & Adolescent Urology, Inc./Akron Children's Hospital, Akron, OH., Pediatric Plastic & Reconstructive Surgery/Akron Children's Hospital, Akron, OH.