Unusual clinical course, Challenging differential diagnosis, Diagnostic / therapeutic accidents, Management of emergency care, Rare disease, Adverse events of drug therapy
Mayssaa Salman, Nabil J. Khoury, Ibarhim Khalifeh, Hussein A. Abbas, Marianne Majdalani, Miguel Abboud, Samar Muwakkit, Hassan El Solh, Raya Saab
Department of Pediatric and Adolescent Medicine, American University of Beirut, Beirut, Lebanon
Am J Case Rep 2013; 14:481-485
Available online: 2013-11-15
Background: Congenital infantile fibrosarcoma (CIF) is a soft-tissue tumor occurring during the first 2 years of life, most commonly in the extremities. CIF is frequently initially misdiagnosed as a vascular tumor, but its association with bleeding and coagulopathy has not been well characterized.
Case Reports: We describe 2 infants with CIF presenting with bleeding and coagulopathy, requiring urgent intervention. Both patients did well; one underwent partial resection followed by chemotherapy, and the other received 2 cycles of chemotherapy followed by gross total resection. We also provide a review of all reported cases of coagulopathy in the setting of CIF in the English literature, uncovering an association that seems to be more prevalent in patients diagnosed in the neonatal period, with associated anemia and thrombocytopenia, and a significant mortality rate.
Conclusions: CIF needs to be considered in the differential diagnosis of vascular congenital tumors, especially when there is evidence of bleeding, anemia, or thrombocytopenia.
Keywords: Congenital infantile fibrosarcoma, bleeding, vascular tumor