BACKGROUND: Fibrosarcoma is a rare tumor in children with limited information on imaging features of these tumors in the literature. OBJECTIVE: To retrospectively review the imaging features of histologically proven congenital infantile fibrosarcoma. MATERIALS AND METHODS: The list of histologically confirmed congenital infantile fibrosarcomas between November 1999 and June 2013 was obtained from the oncology-pathology database. Imaging features and pathology reports of these tumors were reviewed. Patient charts were reviewed and clinical features, management and outcomes were recorded. RESULTS: During the study period, 13 children (9 girls and 4 boys; age range: 0 day-16 months, median age: 2.5 months) with congenital infantile fibrosarcomas were available for complete radiological review. The translocation (t[12;15]) was present in 11/13 (84.6%) and absent in 2/13. Eight/thirteen (61.5%) tumors were located in extremities (5 in lower and 3 in upper), 3/13 in thoracolumbar paraspinal regions, and one each in abdomen and sternocleidomastoid muscle. Imaging features included iso- to hyperintensity on T1-W, hyperintensity on T2-W as compared to skeletal muscles and heterogeneous enhancement. Six (37.5%) tumors showed hemorrhagic components and 2 (15.4%) showed low intensity foci. None of the patients had evidence of regional or distant metastases at diagnosis. Management included surgical resection only (1/13) and combined surgery and chemotherapy (10/13). Overall survival was 100% with a median follow-up of 49.3 months. CONCLUSION: Congenital infantile fibrosarcoma has nonspecific imaging characteristics but should be high on the differential diagnosis in a soft-tissue tumor presenting in infancy, located in an extremity and showing tumoral hemorrhage. Patients have a favorable outcome.
BACKGROUND:Fibrosarcoma is a rare tumor in children with limited information on imaging features of these tumors in the literature. OBJECTIVE: To retrospectively review the imaging features of histologically proven congenital infantile fibrosarcoma. MATERIALS AND METHODS: The list of histologically confirmed congenital infantile fibrosarcomas between November 1999 and June 2013 was obtained from the oncology-pathology database. Imaging features and pathology reports of these tumors were reviewed. Patient charts were reviewed and clinical features, management and outcomes were recorded. RESULTS: During the study period, 13 children (9 girls and 4 boys; age range: 0 day-16 months, median age: 2.5 months) with congenital infantile fibrosarcomas were available for complete radiological review. The translocation (t[12;15]) was present in 11/13 (84.6%) and absent in 2/13. Eight/thirteen (61.5%) tumors were located in extremities (5 in lower and 3 in upper), 3/13 in thoracolumbar paraspinal regions, and one each in abdomen and sternocleidomastoid muscle. Imaging features included iso- to hyperintensity on T1-W, hyperintensity on T2-W as compared to skeletal muscles and heterogeneous enhancement. Six (37.5%) tumors showed hemorrhagic components and 2 (15.4%) showed low intensity foci. None of the patients had evidence of regional or distant metastases at diagnosis. Management included surgical resection only (1/13) and combined surgery and chemotherapy (10/13). Overall survival was 100% with a median follow-up of 49.3 months. CONCLUSION:Congenital infantile fibrosarcoma has nonspecific imaging characteristics but should be high on the differential diagnosis in a soft-tissue tumor presenting in infancy, located in an extremity and showing tumoral hemorrhage. Patients have a favorable outcome.
Authors: G Cecchetto; M Carli; R Alaggio; P Dall'Igna; G Bisogno; G Scarzello; I Zanetti; G Durante; A Inserra; F Siracusa; M Guglielmi Journal: J Surg Oncol Date: 2001-12 Impact factor: 3.454
Authors: Stephan L Waelti; Laurent Garel; Dorothée Dal Soglio; Françoise Rypens; Michael Messerli; Josée Dubois Journal: Pediatr Radiol Date: 2017-09-07