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Clinical management of infantile fibrosarcoma: a retrospective single-institution review
Lalit Parida1, Israel Fernandez-Pineda, John K Uffman
1Department of Surgery, MS 133, St. Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105-3678, USA.
Insights
Infantile fibrosarcoma (IFS) treatment emphasizes non-mutilating surgery. Chemotherapy and radiotherapy are used for unresectable or positive margin cases, with axial sites requiring careful management.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Pediatric Pathology
Background:
- Infantile fibrosarcoma (IFS) is a rare soft-tissue sarcoma affecting infants.
- This study reviews treatment experience for infantile fibrosarcoma.
Purpose of the Study:
- To analyze treatment outcomes and patterns for infantile fibrosarcoma.
- To identify prognostic factors and optimize management strategies for IFS.
Main Methods:
- Retrospective review of 15 patients diagnosed with infantile fibrosarcoma between 1980 and 2009.
- Analysis of surgical interventions, chemotherapy, radiotherapy, and follow-up data.
Main Results:
- Median age at diagnosis was 3 months; common sites included leg, chest wall, and foot.
- 12 patients survived, 3 died (all with axial primary sites and >1 year at diagnosis).
- Complications included nerve, vessel, and muscle loss; local recurrence and lung metastasis occurred.
Conclusions:
- Non-mutilating surgery is the primary treatment for infantile fibrosarcoma.
- Neoadjuvant chemotherapy is recommended for unresectable tumors; adjuvant chemotherapy for positive margins.
- Radiotherapy is reserved for axial primary sites with incomplete resection.
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.
