PDF(Pubmed)
Abstract：
Desmoid tumors are rare, benign, but locally aggressive fibromatoses that pose significant therapeutic challenges, particularly when located in the head and neck region. This report details the case of an extensive cervical desmoid tumor dependent on the levator scapulae muscle and involving the vertebral artery managed through surgical resection and intraoperative navigation. A 45-year-old male presented with a slowly growing cervical mass. Imaging revealed an 83x68x40 mm mass in the right lateral paravertebral space, dependent on the levator scapulae muscle and involving the vertebral artery. Biopsy confirmed a low-grade fusocellular myofibroblastic neoplasm consistent with a desmoid tumor. Given the poor prognosis associated with the symptomatic mass, surgical resection was performed using Brainlab intraoperative navigation (Brainlab, Munich, Germany). The procedure was successful, with preservation of vital structures and no evidence of recurrence postoperatively. Desmoid tumors in the head and neck region, though rare, require precise diagnostic and therapeutic approaches due to their aggressive nature and proximity to critical anatomical structures. The use of intraoperative navigation, in this case, facilitated accurate tumor resection, minimizing damage to surrounding tissues. Pathological analysis revealed a CTNNB1 gene mutation, specifically the S45P variant, which is associated with an increased risk of recurrence. This case highlights the importance of a multidisciplinary approach, incorporating advanced surgical techniques and genetic analysis, in the management of complex desmoid tumors. Intraoperative navigation proved invaluable in achieving successful surgical outcomes, underscoring its potential utility in similar cases. Continued follow-up is essential, given the potential for recurrence associated with desmoid tumors.
摘要：
纤维瘤很少见，良性,但是局部侵袭性纤维瘤病带来了重大的治疗挑战，特别是当位于头部和颈部区域时。本报告详细介绍了一例广泛的颈椎韧带样肿瘤，该肿瘤依赖于提上肩cap肌，并涉及通过手术切除和术中导航管理的椎动脉。一名45岁的男性表现为宫颈肿块缓慢增长。成像显示右侧椎旁间隙有83x68x40mm肿块，依赖于肩胛骨提上肌并累及椎动脉。活检证实低度融合细胞肌纤维母细胞瘤与硬纤维瘤一致。鉴于与症状性肿块相关的不良预后，使用Brainlab术中导航进行手术切除（Brainlab，慕尼黑,德国)。手术成功了,保留重要结构，没有术后复发的证据。头颈部纤维瘤，虽然罕见，需要精确的诊断和治疗方法，由于其侵略性和接近关键的解剖结构。术中导航的使用,在这种情况下,有助于准确切除肿瘤，减少对周围组织的损害。病理分析显示CTNNB1基因突变，特别是S45P变体，这与复发风险增加有关。这个案例突出了多学科方法的重要性,结合先进的外科技术和基因分析，在复杂的硬纤维瘤的治疗中。术中导航在实现成功的手术结果方面被证明是非常宝贵的，强调其在类似情况下的潜在效用。持续的后续行动至关重要，考虑到与硬纤维瘤相关的复发可能性。
