Neurofibromatosis 1 is a relatively rare genetic disease characterized by widespread neurofibromas originating from the peripheral nervous system. Most growths are benign, but some carry a risk of transformation to malignant peripheral nerve sheath tumors. Although these growths can be found anywhere in the body, they are rarely found in the male external genitalia. This report discusses a case of a 25-year-old male patient with neurofibromatosis 1 presenting with a scrotal mass found to have a very large para-testicular intra-scrotal malignant peripheral nerve sheath tumor that required testicle-sparing radical penectomy.
Keywords: Malignant peripheral nerve sheath tumors; Neurofibromatosis type 1; Scrotum; Urologic neoplasms.
Published by Elsevier Inc.