Ballo M T, Zagars G K, Pollack A, Pisters P W, Pollack R A
Department of Radiation Oncology, University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.
J Clin Oncol. 1999 Jan;17(1):158-67. doi: 10.1200/JCO.1999.17.1.158.
To evaluate the therapeutic value of resection and the potential benefits of and indications for adjuvant and definitive radiation therapy for desmoid tumors.
We performed a retrospective review of 189 consecutive cases of desmoid tumor treated with surgical resection, resection and radiation therapy, or radiation therapy alone. Treatment was surgery alone in 122 cases, surgery and radiation therapy in 46, and radiation therapy alone in 21. Median follow-up was 9.4 years.
Overall, 5- and 10-year actuarial relapse rates were 30% and 33%, respectively. Uncorrected survival rates were 96%, 92%, and 87% at 5, 10, and 15 years, respectively. For the patients treated with surgery, the actuarial relapse rates were 34% and 38% at 5 and 10 years, respectively. Among 78 patients with negative margins, the 10-year recurrence rate was 27%, whereas 40 margin-positive patients had a 10-year relapse rate of 54% (P = .003). Tumors located in an extremity also had a poorer prognosis than did those in the trunk. For patients treated with radiation therapy for gross disease, the 10-year actuarial relapse rate was 24%. For patients treated with combined resection and radiation therapy, the 10-year actuarial relapse rate was 25%. The addition of radiation therapy offset the adverse impact of positive margins seen in the surgical group.
Wide local excision with negative pathologic margins is the treatment of choice for most desmoid tumors. Function-sparing resection is appropriate because adjuvant radiation therapy can offset the adverse impact of positive margins. Unresectable disease should be treated with definitive radiation therapy.
评估手术切除的治疗价值以及辅助性和根治性放射治疗对硬纤维瘤的潜在益处和适应证。
我们对189例连续的硬纤维瘤病例进行了回顾性研究，这些病例接受了手术切除、手术切除联合放射治疗或单纯放射治疗。122例仅接受手术治疗，46例接受手术和放射治疗，21例仅接受放射治疗。中位随访时间为9.4年。
总体而言，5年和10年的精算复发率分别为30%和33%。5年、10年和15年的未校正生存率分别为96%、92%和87%。接受手术治疗的患者，5年和10年的精算复发率分别为34%和38%。在78例切缘阴性的患者中，10年复发率为27%，而40例切缘阳性的患者10年复发率为54%（P = 0.003）。位于四肢的肿瘤预后也比躯干肿瘤差。对于因肉眼可见病变接受放射治疗的患者，10年精算复发率为24%。对于接受手术切除联合放射治疗的患者，10年精算复发率为25%。放射治疗的加入抵消了手术组中切缘阳性的不利影响。
大多数硬纤维瘤的治疗选择是切缘阴性的广泛局部切除。保留功能的切除是合适的，因为辅助性放射治疗可以抵消切缘阳性的不利影响。不可切除的病变应采用根治性放射治疗。
