Kazarian Austin G, West Jeremy M, Brown James A, Erickson Bradley A, Gellhaus Paul T
University of Iowa Carver College of Medicine, Iowa City, IA, USA.
Department of Urology, University of Iowa, Iowa City, IA, USA.
Urol Case Rep. 2021 Apr 26;38:101695. doi: 10.1016/j.eucr.2021.101695. eCollection 2021 Sep.
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.
神经纤维瘤病1型是一种相对罕见的遗传性疾病，其特征是起源于周围神经系统的广泛神经纤维瘤。大多数肿瘤是良性的，但有些有转变为恶性周围神经鞘瘤的风险。虽然这些肿瘤可在身体任何部位发现，但很少见于男性外生殖器。本报告讨论了一例25岁患有神经纤维瘤病1型的男性患者，其阴囊肿物被发现为一个非常大的睾丸旁阴囊内恶性周围神经鞘瘤，需要进行保留睾丸的根治性阴茎切除术。
