Management and Recurrence Patterns of Desmoids Tumors: A Multi-institutional Analysis of 211 Patients

Abstract

Background 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. Methods 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. Results 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). Conclusions 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.

Results of surgical treatment in patients with abdominal desmoid fibromas

“…Хирургическое лечение с различными вариантами адъювантного лечения при ДФ передней брюшной стенки являлось «золотым стандартом» не только на протяжении всей истории изучения ДФ, но остается таковым и в на-стоящее время [12].…”

“…В основном рекомендуют хирургическое удаление опухоли в пределах здоровых тка-ней. В ряде случаев прибегают к комбинации лучевой и системной терапии [4][5][6][7][8][9][10][11][12].…”

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results

“…The local recurrence rate is still high after resection alone due to its infiltrative pattern. Some investigators have reported a recurrence rate of 20-90% in literature (9)(10)(11)(12).The reason for the high rate of local relapse is still unclear but surgical resection margin is thought to be the most effective factor. In several studies, it has been reported that positive surgical margin significantly influences local recurrence rates, while others have not demonstrated any association (19)(20)(21).…”

“…Also, they spread along the fascial planes. A high local recurrence rate is reported in several studies (9)(10)(11)(12). Therefore, in the retrospective studies, radiotherapy (RT) has been suggested for its local control benefits.…”

Hepatocellular Cancer Arising From Ectopic Liver Tissue on Diaphragm in Association with Desmoid Mesenteric Tumor

“…Desmoid tumors, that are also called abdominal fibromatosis, are benign tumors which are not seen very often [8], with an estimated incidence of 2-4 per million people per year [9]; the etiology of desmoids tumors is genetic predisposition (in patients with FAP or Gardner syndrome), trauma, prior abdominal surgery and hormonal factors (endogenous levels of estrogens, pregnancy) [10].…”