Cobianchi Lorenzo, Ravetta Valentina, Viera Francesca Torello, Filisetti Claudia, Siri Barbara, Segalini Edoardo, Maestri Marcello, Dominioni Tommaso, Alessiani Mario, Rossi Sandro, Dionigi Paolo
General Surgery 1, Fondazione IRCCS Policlinico San Matteo, Viale Camillo Golgi 19, 27100 Pavia, Italy.
World J Surg Oncol. 2014 Nov 27;12:361. doi: 10.1186/1477-7819-12-361.
Desmoid tumours are benign, myofibroblastic stromal neoplasms common in Gardner's syndrome, which is a subtype of familial adenomatous polyposis characterized by colonic polyps, osteomas, thyroid cancer, epidermoid cysts, fibromas and sebaceous cysts. The primary treatment is surgery, followed by adjuvant radiotherapy, but the local recurrence rate is high, and wide resection can result in debilitating loss of function. We report the case of a 39-year-old man with Gardner's syndrome who had already undergone a total prophylactic colectomy. He developed desmoid tumours localized in the mesenteric root, abdominal wall and dorsal region, which were treated from 2003 through 2013 with several surgical procedures and percutaneous radiofrequency ablation. In 2008 and 2013, RFA was applied under ultrasonographic guidance to two desmoid tumours localized in the dorsal thoracic wall. The outcomes were low-grade pain and one case of superficial skin necrosis, but so far there has been no recurrence of desmoid tumours in these locations. Surgical resection remains the first-line therapy for patients with desmoid tumours, but wide resection may lead to a poor quality of life. Radiofrequency ablation is less invasive and expensive and is a possible therapeutic option for desmoid tumours in patients with Gardner's syndrome.
硬纤维瘤是一种良性肌成纤维细胞性间质肿瘤,常见于加德纳综合征,加德纳综合征是家族性腺瘤性息肉病的一种亚型,其特征为结肠息肉、骨瘤、甲状腺癌、表皮样囊肿、纤维瘤和皮脂腺囊肿。主要治疗方法是手术,术后辅助放疗,但局部复发率高,广泛切除会导致功能丧失,使人虚弱。我们报告一例39岁加德纳综合征男性患者,该患者已接受全预防性结肠切除术。他出现了位于肠系膜根部、腹壁和背部区域的硬纤维瘤,从2003年至2013年通过多次手术和经皮射频消融进行治疗。2008年和2013年,在超声引导下对位于胸壁背部的两个硬纤维瘤进行了射频消融。结果是轻度疼痛和1例浅表皮肤坏死,但到目前为止,这些部位的硬纤维瘤没有复发。手术切除仍然是硬纤维瘤患者的一线治疗方法,但广泛切除可能导致生活质量差。射频消融侵入性较小且费用较低,是加德纳综合征患者硬纤维瘤的一种可能的治疗选择。