Background: Desmoid tumors (DT) represent a group of rare, distinct lesions. There are few published studies examining outcomes and safety of complex reconstruction after DT resection.
Methods: A retrospective review identified 39 patients who underwent surgical treatment of DT at St. Jude Children's Research Hospital over a 12-year period. A systematic review of the literature identified 17 further studies for inclusion. Treatment characteristics were analyzed.
Result: Thirty-nine patients were treated during the study period, with a total number of 67 resections. Median age was 12.2 years; 49% of patients were male, and 51% were female. Median tumor size was 9.8 cm. DT most commonly arose in the extremities (40%), thorax (23%), head and neck (21%), and trunk (16%). One- and 5-year recurrence-free survival were 97.1% and 73.1%, respectively. The majority of defects were closed primarily, with the exception of head and neck defects. Long-term outcomes were good for chest, abdomen, and upper extremity defects, but were problematic for head and neck, breast, and lower extremity defects. There were no recurrences at the site of flap harvest in either the study population or in reviewed studies.
Conclusion: For patients with DT, surgical extirpation should not come at the expense of functional preservation, as overall survival is excellent. However, specific defects, including those of the lower extremity, breast, and head and neck, will benefit from improved techniques for resection and reconstruction.