gms | German Medical Science

Background: Solitary fibrous tumors (SFT) represent a rare entity of soft tissue tumors. Previously considered beeing of serosal origin and solely limited to the pleural cavity the tumor recently has been described in other locations, especially the head and neck. Extrathoracic SFT in the soft tissues of the trunk and the extremities are very rare. Nine cases of this rare tumor entity are described with respect to clinicopathological data, follow-up and treatment results.

Methods: Data was obtained from patients records, phone calls to the patients general practitioners, and clinical follow up examination, chest X-ray and abdominal ultra sound.

Results: There were 6 females and 3 males, whose age at time of diagnosis ranged from 32 to 92 years (mean: 62.2 years). The documented tumors’ size was 4.5 to 6 cm (mean: 7 cm). All tumors were located in deep soft tissues, 3 of them epifascial, 2 subfascial, 4 intramuscular. Four tumors were found at the extremities, one each at the flank, in the neck, at the shoulder, in the gluteal region, and in the deep groin. Two out of 9 cases were diagnosed as atypical or malignant variant of ESFT. Complete resection was performed in all cases. Follow-up time ranged from 1 to 71 months, 1 same patient with atypical ESFT suffered from local relapse and metastatic disease, 8 patients were free of disease.

Conclusions: ESFT usually behave as benign soft tissue tumors although malignant variants with more aggressive local behaviour (local relapse) and metastasis may occur. The risk of local recurrence and metastasis correlates to tumor size and histological status of surgical resection margins and may reach up to 10% even in so called “benign” tumors. Tumor specimens should be evaluated by experienced soft tissue pathologists. The treatment of choice is complete resection followed by extended follow-up surveillance.