Function-preserving surgery in sporadic desmoid-type fibromatosis of brachial plexus.

Sundarakrishnan Dharanipathy, Amol Raheja, Sachin Anil Borkar, Ashish Suri, Rimlee Dutta, Aruna Nambirajan, Shiv Kumar Choudhary, Pradeep Ramakrishnan, Bhavuk Garg, Sameer Rastogi
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Abstract

Desmoid tumours of the brachial plexus are rare locally infiltrative aggressive, monoclonal, fibroblastic proliferations characterized by a variable and often unpredictable clinical course. Only 21 patients have been reported in the literature. We add another one, and report function-preserving surgery in a 34-year-old man with a desmoid tumour of the brachial plexus. The patient presented with paraesthesia and gradually progressive distal muscle weakness in the left upper limb. Electrodiagnostic studies revealed preganglionic changes in segments C8-D1. Contrast-enhanced magnetic resonance imaging showed an enhancing mass with irregular margins in the left paravertebral region encasing the subclavian artery, pre- and post-ganglionic C6-D1 nerve roots and trunks of the brachial plexus. Using an anterior transclavicular approach the tumour was decompressed, which led to a major improvement in paraesthesia and partial motor recovery. He was doing well at 6 months of follow-up. Histopathological examination showed findings consistent with desmoid tumour. A tailored multidisciplinary surgical approach, with the aim to preserve function over radiological clearance, is an acceptable treatment strategy in preserving patient's quality of life for such infiltrating desmoid tumours encasing the brachial plexus. Following surgery, observation and close radiological surveillance offer an optimal strategy without jeopardizing the quality of life.

臂丛神经散发性去瘤型纤维瘤病的保留功能手术。
臂丛蝶形瘤是一种罕见的局部浸润性侵袭性、单克隆、纤维母细胞增生性肿瘤,其特点是临床病程多变且往往难以预测。文献中仅报道过 21 例患者。我们又增加了一名患者,并报告了一名患有臂丛干酪样瘤的 34 岁男性的保留功能手术。患者出现左上肢麻痹和逐渐进展的远端肌无力。电诊断研究显示,C8-D1节段出现神经节前改变。对比增强磁共振成像显示,左侧椎旁区域有一个边缘不规则的强化肿块,包绕锁骨下动脉、C6-D1节段前后神经根和臂丛神经干。采用经锁骨前入路对肿瘤进行了减压,从而大大改善了麻痹症状,部分运动功能也得到了恢复。随访6个月后,他的情况良好。组织病理学检查结果显示与类苔藓样肿瘤一致。对于这种包裹臂丛神经的浸润性类脂样瘤,采用量身定制的多学科手术方法是一种可接受的治疗策略,其目的是保护功能而非放射学清除,以保持患者的生活质量。手术后,观察和密切的放射学监测是不影响生活质量的最佳策略。
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