Ricciardelli Ashley, Flores Alex, Lu Hsiang-Chih, Guney Ekin, Perry Arie, Grochmal Joey
Departments of1Neurosurgery.
2Pathology and Immunology, Baylor College of Medicine, Houston, Texas.
J Neurosurg Case Lessons. 2024 Jan 29;7(5). doi: 10.3171/CASE23517.
Neuromuscular choristomas (NMCs) are rare tumors involving aberrant intercalation of muscle fibers into peripheral nerves, most commonly the sciatic nerve. Although benign, these lesions risk developing into NMCs with desmoid-type fibrosis (NMC-DTFs), aggressive lesions potentially requiring amputation. Currently, information on NMCs and the link between NMCs and NMC-DTFs is limited in adults, with the majority of cases reported in children. We present the case of a 66-year-old male with a sciatic NMC alongside a Preferred Reporting Items for Systematic Reviews and Meta-Analyses-based systematic review of similar cases to better characterize this lesion in the adult population.
A male presented with 10 years of progressive left lower-extremity weakness and paresthesia, and a mildly enlarged proximal sciatic nerve was discovered on magnetic resonance imaging. He underwent left sciatic fascicular nerve biopsy, with histopathological examination identifying the lesion as an NMC. The literature review revealed that our case, alongside other cases of adults with NMCs, shared characteristics similar to NMCs in the pediatric population.
More comprehensive studies of adults with NMCs are needed, as the current literature contains limited information concerning disease course, diagnostic characteristics, and treatment. Furthermore, NMCs in adults should be handled with care because of the increased likelihood of transformation to NMC-DTF after surgical intervention.
神经肌肉迷离瘤(NMCs)是一种罕见肿瘤,其特征是肌纤维异常插入周围神经,最常见于坐骨神经。尽管这些病变是良性的,但有发展为伴有硬纤维瘤样纤维化的神经肌肉迷离瘤(NMC-DTFs)的风险,这种侵袭性病变可能需要截肢。目前,关于成人NMCs以及NMCs与NMC-DTFs之间联系的信息有限,大多数病例报告见于儿童。我们报告一例66岁男性坐骨神经NMC病例,并基于系统评价和Meta分析的首选报告项目对类似病例进行系统评价,以更好地描述成人患者中的这种病变。
一名男性出现进行性左下肢无力和感觉异常10年,磁共振成像发现近端坐骨神经轻度增粗。他接受了左侧坐骨神经束活检,组织病理学检查将病变确诊为NMC。文献综述显示,我们的病例以及其他成人NMC病例与儿童NMCs具有相似特征。
需要对成人NMCs进行更全面的研究,因为目前的文献中关于疾病病程、诊断特征和治疗的信息有限。此外,由于手术干预后成人NMCs转变为NMC-DTF的可能性增加,因此对其处理应谨慎。