Congenital infantile fibrosarcoma: review of imaging features.
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.


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.


CONCLUSIONS
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.
Department of Diagnostic Imaging, McMaster University Medical Centre and McMaster University, Hamilton, ON, Canada.
Kelly E
KE
Ainsworth
Govind B
GB
Chavhan
Abha A
AA
Gupta
Sevan
S
Hopyan
Glenn
G
Taylor
D003287:Contrast Media; D003952:Diagnostic Imaging; D005260:Female; D005354:Fibrosarcoma / Q000151:congenital* / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012983:Soft Tissue Neoplasms / Q000151:congenital* / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D016896:Treatment Outcome
D003287:Contrast Media