OBJECTIVES: Extrathoracic solitary fibrous tumors (ESFTs) are rare low-to-intermediate grade spindle-cell neoplasms of pluripotent fibroblastic or myofibroblastic origin. This review explores prognostic factors in the management of ESFTs and provides guidance on optimal treatment regimens based on the current literature. PATIENTS AND METHODS: Electronic searches were performed using MEDLINE, Embase, and the Cochrane library to identify studies on prognostic factors in the management of ESFTs published between January 1970 and June 2016. The literature search and review process identified 100 articles that were included in this review article. This included both surgical and nonsurgical studies on the management of ESFTs. RESULTS: Surgical excision with wide resection margins forms the mainstay of treatment and provides optimal long-term oncological outcomes. Large tumor size (>5 to 10 cm diameter), inadequate resection margins, malignant histologic features, dedifferentiation, and tumor location within the abdomen/pelvis are associated with adverse oncological outcomes. Radiotherapy may be used for preoperative tumor shrinkage and/or as adjuvant therapy in patients with malignant disease or incomplete surgical margins. Chemotherapy with molecular-targeted therapies has produced promising results and the results of further phase 2 trials are awaited. CONCLUSIONS: Routine long-term follow-up is essential for benign and malignant disease to enable early detection and treatment of recurrent disease.
OBJECTIVES: Extrathoracic solitary fibrous tumors (ESFTs) are rare low-to-intermediate grade spindle-cell neoplasms of pluripotent fibroblastic or myofibroblastic origin. This review explores prognostic factors in the management of ESFTs and provides guidance on optimal treatment regimens based on the current literature. PATIENTS AND METHODS: Electronic searches were performed using MEDLINE, Embase, and the Cochrane library to identify studies on prognostic factors in the management of ESFTs published between January 1970 and June 2016. The literature search and review process identified 100 articles that were included in this review article. This included both surgical and nonsurgical studies on the management of ESFTs. RESULTS: Surgical excision with wide resection margins forms the mainstay of treatment and provides optimal long-term oncological outcomes. Large tumor size (>5 to 10 cm diameter), inadequate resection margins, malignant histologic features, dedifferentiation, and tumor location within the abdomen/pelvis are associated with adverse oncological outcomes. Radiotherapy may be used for preoperative tumor shrinkage and/or as adjuvant therapy in patients with malignant disease or incomplete surgical margins. Chemotherapy with molecular-targeted therapies has produced promising results and the results of further phase 2 trials are awaited. CONCLUSIONS: Routine long-term follow-up is essential for benign and malignant disease to enable early detection and treatment of recurrent disease.