Clinical management of infantile fibrosarcoma: a retrospective single-institution review.

BACKGROUND

Infantile fibrosarcoma (IFS) is an uncommon soft-tissue sarcoma. Here we review our experience treating this tumor.

PATIENTS AND METHODS

We retrospectively reviewed records of patients with IFS treated at St. Jude Children's Research Hospital between 1980 and 2009.

RESULTS

We identified 15 patients, 8 girls and 7 boys; 13 white and 2 black. Median age at diagnosis was 3 months. Primary sites included the leg (n = 3), chest wall (n = 2), foot (n = 2), and one each in the tongue, occipital region, axilla, parascapular region, arm, forearm, retroperitoneum, and thigh. All patients underwent resection; 11 upfront surgery, and 4 delayed. Complications included loss of the posterior tibial nerve and artery, axillary vein, biceps, pectoralis major, gallbladder, and transverse/sigmoid sinus. Eight received chemotherapy and three radiotherapy. Seven experienced local recurrence and three lung metastasis. Median follow-up was 65 months. At the time of the review, 12 patients were alive and 3 had died. All deaths were in patients older than 1 year at diagnosis with an axial primary site.

CONCLUSIONS

Non-mutilating surgery should be the primary treatment for IFS. Neoadjuvant chemotherapy is indicated when upfront resection is unfeasible. Patients with positive surgical margins should receive adjuvant chemotherapy. Radiotherapy is indicated for axial primary sites where complete resection is impossible.