Congenital fibrosarcoma of the ileum: case report with molecular confirmation and literature review.
AffiliationDepartment of Pathology, Our Lady's Children's Hospital, Crumlin, Dublin,, Ireland.
MeSHChromosomes, Human, Pair 12/genetics
Chromosomes, Human, Pair 15/genetics
Oncogene Proteins, Fusion/genetics
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CitationFetal Pediatr Pathol. 2011;30(3):156-60. Epub 2011 Feb 28.
JournalFetal and pediatric pathology
AbstractCongenital fibrosarcoma is a rare, soft tissue malignancy of infancy, most commonly involving the distal extremities. We report a case of congenital fibrosarcoma of the ileum in a 5-day-old boy who presented with an acute abdomen due to ileal perforation. Partial ileal resection was carried out with successful anastomosis. Grossly, the resected small bowel showed focal luminal stenosis with a thickened, indurated wall. Histology showed a transmural primitive spindle cell proliferation with a morphology consistent with congenital fibrosarcoma. The associated hallmark chromosomal translocation t(12;15)(q13;q25) was demonstrated by reverse transcriptase polymerase chain reaction.
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