Congenital infantile fibrosarcoma: Association with bleeding diathesis
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Mayssaa Salman1, Nabil J Khoury, Ibrahim Khalifeh
1Department of Pediatric and Adolescent Medicine, American University of Beirut, Beirut, Lebanon.
Insights
Congenital infantile fibrosarcoma (CIF) can present with bleeding and coagulopathy, often mimicking vascular tumors. Early consideration of CIF is crucial, especially with anemia or thrombocytopenia, to improve patient outcomes.
Area of Science:
- Pediatric Oncology
- Hematology
- Oncology
Background:
- Congenital infantile fibrosarcoma (CIF) is a rare soft-tissue tumor affecting infants, often misdiagnosed as vascular tumors.
- The association between CIF and bleeding or coagulopathy is not well-established.
Purpose of the Study:
- To describe cases of CIF presenting with bleeding and coagulopathy.
- To review the literature for reported cases of coagulopathy in CIF.
- To highlight the importance of considering CIF in the differential diagnosis of congenital tumors with bleeding complications.
Main Methods:
- Case report of two infants with CIF, bleeding, and coagulopathy.
- Systematic literature review of coagulopathy associated with CIF.
- Analysis of patient demographics, clinical presentation, treatment, and outcomes.
Main Results:
- Two infants with CIF presented with bleeding and coagulopathy, requiring intervention and responding well to treatment (surgery and chemotherapy).
- Literature review revealed a higher prevalence of coagulopathy in neonatal CIF cases, often with anemia and thrombocytopenia.
- A significant mortality rate was observed in CIF patients with coagulopathy.
Conclusions:
- Congenital infantile fibrosarcoma should be included in the differential diagnosis of congenital vascular tumors, particularly when bleeding, anemia, or thrombocytopenia are present.
- Prompt recognition and management of coagulopathy in CIF are critical for improving survival rates.
Patient:
Male, 2 month
Final Diagnosis:
Congenital infantile fibrosarcoma Symptoms: Bleeding Medication: Vincristine • actinomycin • cyclophosphamide Clinical Procedure: Surgical resection Specialty: Pediatric Oncology.
Objective:
Diagnostic/therapeutic accidents.
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.
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