Maldonado Andres A, Broski Stephen M, Carter Jodi M, Spinner Robert J
Departments of1Neurologic Surgery and.
2Radiology, Mayo Clinic, Rochester, Minnesota; and.
J Neurosurg Case Lessons. 2023 Feb 6;5(6). doi: 10.3171/CASE22554.
Neuromuscular choristoma (NMC) is a rare congenital lesion in which muscle tissue is admixed with nerve fascicles within a peripheral nerve. Patients commonly present in early childhood with neuropathy, plexopathy, or chronic undergrowth in the distribution of the affected nerve.
The authors present the case of a 35-year-old man with unrecognized neuromuscular NMC of the sciatic nerve, which resulted in recurrent, multicentric NMC-associated desmoid-type fibromatosis (NMC-DTF) within the nerve territory in association with a Marjolin ulcer, a cutaneous malignancy.
Based on anatomical and pathophysiological findings described in this case report, the authors support the association between NMC-DTF and Marjolin ulcer.
神经肌肉错构瘤(NMC)是一种罕见的先天性病变,其中肌肉组织与周围神经内的神经束混合存在。患者通常在儿童早期出现受影响神经分布区域的神经病变、丛病变或慢性发育不良。
作者报告了一例35岁男性,患有未被识别的坐骨神经神经肌肉NMC,该病变导致神经区域内反复出现多中心的NMC相关硬纤维瘤病(NMC-DTF),并伴有皮肤恶性肿瘤马乔林溃疡。
基于本病例报告中描述的解剖学和病理生理学发现,作者支持NMC-DTF与马乔林溃疡之间的关联。