Department of Urology, Cleveland Clinic Akron General, Akron, OH.
Department of Urology, Cleveland Clinic Akron General, Akron, OH.
Urology. 2020 Jan;135:133-135. doi: 10.1016/j.urology.2019.09.028. Epub 2019 Oct 2.
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.
恶性外周神经鞘瘤（MPNST）是一种起源于外周神经纤维的软组织肉瘤，主要发生在神经纤维瘤病（NF1）的背景下。发生于阴茎的 MPNST 非常罕见，可能需要进行致残性手术才能实现手术治愈。我们之前曾报道过一例 14 个月大的男孩患有阴茎 MPNST 的病例，该患者接受了新辅助化疗、全阴茎切除术和辅助放疗。在此，我们报告了同一患者的中期随访结果，描述了他随后的泌尿生殖系统重建，并讨论了阴茎 MPNST 治疗后出现的管理难题。
