Boman F, Palau C, Floquet A, Floquet J, Lascombes P
Service d'Anatomie Pathologique, Hôpital de Brabois, Faculté de Médecine, Vandoeuvre-Les-Nancy.
Ann Pathol. 1991;11(1):36-41.
Neuromuscular hamartoma is a pseudotumour characterized by the presence of striated muscle fibers in a nerve trunk. The reported case was located in the left sciatic nerve in a 4-year-old girl with equinism and spasticity. A first surgical procedure, with muscle biopsy, did not permit diagnosis of hamartoma. It was followed by the occurrence of a firm mass at the same location. Partial excision was performed; the mass revealed to be fibrous. Further aggravation of functional symptoms lead to leg amputation and correct diagnosis. Discussion is focused on the unique features of this rare disorder, for which therapeutic attempts might be avoided, whereas diagnosis proved to be sometimes difficult.
神经肌肉错构瘤是一种假瘤,其特征是神经干中存在横纹肌纤维。报告的病例位于一名患有马蹄足和痉挛的4岁女孩的左侧坐骨神经。首次手术及肌肉活检未能诊断出错构瘤。随后在同一位置出现了一个硬结。进行了部分切除;肿块显示为纤维性。功能症状进一步加重导致截肢并确诊。讨论集中在这种罕见疾病的独特特征上,对于这种疾病可能应避免进行治疗尝试,而诊断有时被证明是困难的。