PDF(Pubmed)
Abstract：
Desmoid tumors are locally aggressive, benign neoplasms originating in connective tissues. Although the exact pathophysiology remains unknown, antecedent trauma or surgery are believed to be important contributing factors. The occurrence of paraspinal desmoid tumor in pediatric patients is extremely uncommon. Here, we present an exceedingly rare case of a pediatric patient with no surgical or family history who developed a paraspinal desmoid tumor. A 9-year-old female patient presented with 4 months of progressive back pain, right lower extremity weakness, and numbness. Spinal imaging revealed a left epidural paraspinal mass compressing her thoracic spinal cord and extending into the left thoracic cavity. A multidisciplinary approach with neurosurgery and thoracic surgery enabled gross total resection of the lesion. The patient had complete resolution of her symptoms with no signs of residual tumor on postoperative imaging. Pathology revealed a desmoid tumor that avidly stained for beta-catenin. On her last follow-up, she developed a recurrence, to which she was started on sorafenib therapy. Desmoid tumors are rare connective tissue neoplasms that often occur after local tissue trauma, such as that caused by surgery. This report presents a rare case of a pediatric paraspinal desmoid tumor that occurred in a patient with no surgical or family history. Such tumors should undergo surgical resection for symptomatic relief and tissue diagnosis. Close clinical and radiographic surveillance are essential in these patients due to the high recurrence rates of desmoid tumor.
摘要：
纤维瘤是局部侵袭性的，起源于结缔组织的良性肿瘤。尽管确切的病理生理学仍然未知，先前的创伤或手术被认为是重要的促成因素。儿童患者的椎旁硬纤维瘤的发生极为罕见。这里,我们介绍了一例极为罕见的病例,其中一例没有手术史或家族史的儿科患者发生了椎旁硬纤维瘤.一名9岁女性患者出现4个月的进行性背痛，右下肢无力,和麻木。脊柱成像显示左侧硬膜外椎旁肿块压迫了她的胸脊髓并延伸到左胸腔。神经外科和胸外科的多学科方法使病灶完全切除。患者的症状完全缓解，术后影像学无残留肿瘤迹象。病理学显示，硬纤维状肿瘤被β-连环蛋白染色。在她的最后一次随访中，她复发了,她开始接受索拉非尼治疗。纤维瘤是罕见的结缔组织肿瘤，常发生在局部组织创伤后，比如手术引起的。本报告介绍了一例罕见的小儿椎旁硬纤维瘤，该病例发生在无手术史或家族史的患者中。此类肿瘤应进行手术切除以缓解症状和进行组织诊断。由于硬纤维瘤的高复发率，因此对这些患者进行密切的临床和影像学监测至关重要。
