Infantile fibrosarcoma: Clinical and histologic responses to cytotoxic chemotherapy
- 1 April 2009
- journal article
- case report
- Published by Wiley in Pediatric Blood & Cancer
- Vol. 53 (1), 23-27
- https://doi.org/10.1002/pbc.21981
Abstract
Background Infantile fibrosarcoma (IF) is a rare soft tissue sarcoma that presents either at birth or in the first year of life. Complete surgical resection is usually curative but chemotherapy may shrink the tumor to facilitate complete resection. This report describes the histologic changes and outcomes in four patients with IF treated with chemotherapy and surgical resection. Procedure A retrospective review was performed of patients treated between 2000 and 2007. Results All four patients are alive with excellent functional outcomes. The patients were diagnosed from birth up to 7 months of age; three had lower extremity tumors and one had a neck tumor. All patients received vincristine, cyclophosphamide, and actinomycin; one patient also received ifosfamide and etoposide after tumor progression. One tumor, arising from the neck, had rapid shrinkage. Two lower extremity tumors had only modest changes in dimensions but upon resection, the tumor bed contained fibrous tissue with exaggerated small caliber vessels. The fourth infant developed metastatic lesions in the central nervous system, orbits, lungs, and kidney after complete removal of the primary tumor. The metastatic lesions responded to chemotherapy and have remained stable for over 3 years. Conclusions IF is a chemosensitive tumor. In patients where a clinical response is not apparent, cytoreduction of the tumor and replacement with fibrotic and fibrovascular tissue may facilitate gross‐total resection. The chemotherapy‐responsiveness of this tumor may abrogate unfavorable features such as metastatic or residual tumor. Pediatr Blood Cancer 2009;53:23–27.This publication has 13 references indexed in Scilit:
- Surgical Risk Factors in Primary Surgery for Localized Neuroblastoma: The LNESG1 Study of the European International Society of Pediatric Oncology Neuroblastoma GroupJournal of Clinical Oncology, 2005
- Congenital Fibrosarcoma with Metastasis in a FetusPediatric and Developmental Pathology, 2004
- Retroperitoneal Infantile Fibrosarcoma: Clinical, Molecular, and Therapeutic Aspects of an Unusual TumorPediatric Hematology and Oncology, 2003
- Treatment of Infantile Fibrosarcoma With Chemotherapy and Surgery: Results From the Dana-Farber Cancer Institute and Children's Hospital, BostonJournal of Pediatric Hematology/Oncology, 2002
- Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors: congenital (infantile) fibrosarcoma and mesoblastic nephromaCancer Genetics and Cytogenetics, 2002
- Congenital Mesoblastic Nephroma t(12;15) Is Associated withETV6-NTRK3 Gene Fusion: Cytogenetic and Molecular Relationship to Congenital (Infantile) FibrosarcomaThe American Journal of Pathology, 1998
- Congenital mesoblastic nephroma metastatic to the brain: a report of two casesPediatric Radiology, 1995
- Chemotherapy in the management of infantile fibrosarcomaMedical and Pediatric Oncology, 1993
- Congenital Fibrosarcoma Metastatic To the ChoroidAmerican Journal of Ophthalmology, 1979
- Infantile fibrosarcomaCancer, 1976