Smith A J, Lewis J J, Merchant N B, Leung D H, Woodruff J M, Brennan M F
Departments of Surgery, Biostatistics and Pathology, Memorial Sloan-Kettering Cancer Center, New York, USA.
Br J Surg. 2000 May;87(5):608-13. doi: 10.1046/j.1365-2168.2000.01400.x.
Intra-abdominal desmoids are uncommon neoplasms. The aggressive nature of these tumours and the potential for major morbidity secondary to resection can present a difficult surgical dilemma.
Patients with histologically confirmed intra-abdominal desmoid tumours undergoing laparotomy were identified from a prospective database. Clinical features and outcomes in this group were evaluated.
The study group comprised 24 patients. Sixteen patients underwent complete resection of the tumour while eight had biopsy only, with or without intestinal bypass. Small intestinal resection was performed in 12 patients, including three who had a near-total enterectomy. Median follow-up was 62 months, with an actuarial overall survival rate of 73 per cent at 10 years. There was no difference in survival rate between completely resected and unresected patients (P = 0.73). There were seven deaths in the entire group, of which four were in those undergoing complete resection.
Operation can cure patients with intra-abdominal desmoid tumours, but may result in significant morbidity, especially from loss of small intestine. No other therapy is a predictably good alternative to operation but the natural history of desmoids is often characterized by prolonged periods of stability or even regression. A period of watchful waiting, until significant symptoms develop, may be the most appropriate course in patients who risk mesenteric vascular injury or substantial enterectomy with attempts at resection.
腹腔内硬纤维瘤是罕见的肿瘤。这些肿瘤的侵袭性以及切除术后继发严重并发症的可能性,会带来棘手的手术难题。
从一个前瞻性数据库中识别出经组织学确诊为腹腔内硬纤维瘤且接受剖腹手术的患者。对该组患者的临床特征和预后进行评估。
研究组包括24例患者。16例患者接受了肿瘤的完整切除,8例仅进行了活检,部分伴有或不伴有肠道旁路手术。12例患者进行了小肠切除术,其中3例进行了近全小肠切除术。中位随访时间为62个月,10年的精算总生存率为73%。完整切除组和未切除组的生存率无差异(P = 0.73)。全组有7例死亡,其中4例发生在接受完整切除的患者中。
手术可治愈腹腔内硬纤维瘤患者,但可能导致严重并发症,尤其是小肠缺失。没有其他治疗方法可作为手术的可预测的良好替代方案,但硬纤维瘤的自然病程通常以长期稳定甚至消退为特征。对于有肠系膜血管损伤风险或试图切除时可能进行大量肠切除的患者,在出现明显症状之前进行一段时间的观察等待可能是最合适的做法。