Management and recurrence patterns of desmoids tumors: a multi-institutional analysis of 211 patients.

Management and recurrence patterns of desmoids tumors: a multi-institutional analysis of 211 patients.
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DOI:
10.1245/s10434-012-2634-6
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发表时间:
2012-12
影响因子:
3.7
通讯作者:
Pawlik TM
Pawlik TM
中科院分区:
医学2区
文献类型:
--
作者:
Peng PD;Hyder O;Mavros MN;Turley R;Groeschl R;Firoozmand A;Lidsky M;Herman JM;Choti M;Ahuja N;Anders R;Blazer DG 3rd;Gamblin TC;Pawlik TM

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硬纤维瘤是一种罕见的软组织肿瘤,其治疗资料有限。我们试图确定硬纤维瘤手术后的复发率,并确定预测无病生存的因素。1983年1月至2011年12月,211例硬纤维瘤患者来自三个主要的外科中心。分析临床病理和治疗特点,以确定复发的预测因素。中位年龄36岁;患者以女性为主(68%)。硬纤维瘤最常见于四肢(32%)、腹腔(23%)或腹壁(21%)和胸腔(15%);中位尺寸为7.5厘米。大多数患者手术切缘为R0(60%)。1年和5年无复发生存率分别为81.3%和52.8%。与无复发生存率较差相关的因素有:年龄更年轻(年龄每增加5年,风险比[HR] = 0.90, 95%可信区间[95% CI] 0.82 ~ 0.98)和腹外肿瘤位置(腹壁参照:腹外位置,HR = 3.28, 95% CI, 1.46 ~ 7.36)(均P <0.05)。硬纤维瘤切除术后复发仍然是一个问题,多达50%的患者在5年内复发。与复发相关的因素包括年龄、肿瘤位置和边缘状态。虽然手术切除仍然是硬纤维瘤患者治疗的核心,但高复发率强调需要更有效的辅助治疗。
Desmoid tumors are rare soft-tissue neoplasms with limited data on their management. We sought to determine the rates of recurrence following surgery for desmoid tumors and identify factors predictive of disease-free survival. Between January 1983 and December 2011, 211 patients with desmoid tumors were identified from three major surgical centers. Clinicopathologic and treatment characteristics were analyzed to identify predictors of recurrence. Median age was 36 years; patients were predominantly female (68 %). Desmoid tumors most commonly arose in extremities (32 %), abdominal cavity (23 %) or wall (21 %), and thorax (15 %); median size was 7.5 cm. Most patients had an R0 surgical margin (60 %). The 1- and 5-year recurrence-free survival was 81.3 and 52.8 %, respectively. Factors associated with worse recurrence-free survival were: younger age (for each 5-year increase in age, hazard ratio [HR] = 0.90, 95 % confidence interval [95 % CI] 0.82–0.98) and extra-abdominal tumor location (abdominal wall referent: extra-abdominal site, HR = 3.28, 95 % CI, 1.46–7.36) (both P <0.05). Recurrence remains a problem following resection of desmoid tumors with as many as 50 % of patients experiencing a recurrence within 5 years. Factors associated with recurrence included age, tumor location, and margin status. While surgical resection remains central to the management of patients with desmoid tumors, the high rate of recurrence highlights the need for more effective adjuvant therapies.
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