Almesned Razan, Alhagbani Mohamed, Sultan Mohammed, Alshayie Mohammed, Alqarni Naif, Alshammari Ahmed
Department of Urology, Pediatric Urology Division, King Faisal Specialists Hospital and Research Center, Riyadh, Saudi Arabia.
Department of Urology, Pediatric Urology Division, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
Urol Ann. 2023 Jul-Sep;15(3):328-330. doi: 10.4103/ua.ua_86_22. Epub 2023 Jul 17.
Neurofibromatosis of the genitourinary tract is rare, with a prevalence of 0.65%, and it is exceedingly rare to involve the external genitalia. Involvement of the clitoris, labia majora, and prepuce was reported with clitoromegaly being the most frequently occurring. Herein, we are reporting the case of a 6-year-old girl who was diagnosed with a neurofibroma of the clitoris; measuring 9.4 cm in its largest dimension. To the best of our knowledge, this is the largest clitoral neurofibroma reported in the literature. Due to the rarity of such cases and reports limitations in the literature, the diagnosis of neurofibroma of the external genitalia requires a high index of suspicion by health-care providers. Surgical excision and postoperative follow-up for possible recurrence remain the gold standard of management.
泌尿生殖道神经纤维瘤病很罕见,患病率为0.65%,累及外生殖器极为罕见。曾有阴蒂、大阴唇和包皮受累的报道,其中阴蒂肥大最为常见。在此,我们报告一例6岁女孩被诊断为阴蒂神经纤维瘤的病例;其最大直径为9.4厘米。据我们所知,这是文献中报道的最大的阴蒂神经纤维瘤。由于此类病例罕见且文献报道有限,医疗保健人员对外生殖器神经纤维瘤的诊断需要高度怀疑。手术切除及术后对可能复发情况的随访仍是治疗的金标准。