Abstract
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.
Highlights
Desmoid tumors are rare soft-tissue neoplasms with limited data on their management
Recurrence remains a problem following resection of desmoid tumors with as many as 50 % of patients experiencing a recurrence within 5 years
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
Summary
Between January 1983 and December 2011, 211 patients with desmoid tumors were identified from three major surgical centers. Using a multi-institutional database, patients with histologically proven desmoid tumors at 1 of 3 institutions (Johns Hopkins School of Medicine, Baltimore, MD; Duke Medical Center, Durham, NC; Medical College of Wisconsin, Milwaukee, WI) between January 1983 and December 2011 were identified. Patients with histologically confirmed primary desmoid tumors who received their treatment at a study center were included. Data were collected on primary desmoid tumor location and size. Date of last follow-up, vital status, and recurrence-related information were collected on all patients. Information regarding the location, as well as the disease-free interval from the date of initial operation to the development of recurrent disease was recorded. Dates of last follow-up and vital status were collected on all patients
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