Zhao Jiming, Cheng Fajuan, Yao Zhigang, Zheng Bin, Niu Zhihong, He Wei
Department of Urology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, China.
Department of Urology, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, China.
Front Surg. 2022 Apr 11;9:851164. doi: 10.3389/fsurg.2022.851164. eCollection 2022.
Desmoid fibromatosis (DF) is a rare clonal proliferation of fibroblasts and myofibroblasts. It develops in the connective tissues and does not metastasize but may infiltrate adjacent structures. Because of the rarity of these tumors and the unpredictable natural history of the disease, well-defined and precise guidelines of the optimal treatment for DF have not been formulated.
Here, we present a giant abdominal DF that invaded the right spermatic cord and iliac vessels. The lesion was excised with external iliac artery dissection; however, the vein was sacrificed. The abdominal wall defect was then repaired with a polypropylene mesh. The lesional cells are positive for β-catenin.
In the past decades, there has been a change in the treatment of DF. The "wait and see" policy has been considered initially in most cases. Surgical intervention remains a valid option for symptomatic lesions. The optimal regimes of the tumor should not take the risk of making the patient more symptomatic than the lesion itself.
韧带样纤维瘤病（DF）是一种罕见的成纤维细胞和肌成纤维细胞的克隆性增殖疾病。它发生于结缔组织，不发生转移，但可能浸润邻近结构。由于这些肿瘤罕见且疾病自然史不可预测，尚未制定出明确且精确的DF最佳治疗指南。
在此，我们报告一例巨大的腹部DF，其侵犯了右侧精索和髂血管。病变通过髂外动脉剥离术切除；然而，静脉被牺牲。然后用聚丙烯网片修复腹壁缺损。病变细胞β-连环蛋白呈阳性。
在过去几十年中，DF的治疗发生了变化。大多数情况下最初会考虑“观察等待”策略。手术干预对于有症状的病变仍然是一种有效的选择。肿瘤的最佳治疗方案不应使患者出现比病变本身更严重的症状。
