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Kaposiform Hemangioendothelioma in a 3 Months Old Infant
Ramya Uppuluri1, Vimal Kumar1, Deenadayalan Munirathnam1
1Department of Pediatric Hematology, Oncology and BMT, Apollo Speciality Hospital, 320, Padma Complex, Anna Salai, Chennai, 600035 India.
Abstract:
Kaposiform hemangioendothelioma is a rare, highly vascular and aggressive soft tissue tumor frequently associated with Kasabach-Merritt phenomenon, usually seen in early infancy. Early diagnosis by means of MRI and tissue biopsy portends a better outcome. Treatment includes surgical excision when feasible and medical management with steroids, propranolol, vincristine and supportive treatment for coagulopathy. We report a 3 months old female infant who was diagnosed, treated successfully and is now in complete remission.
Insights
Kaposiform hemangioendothelioma is a rare vascular tumor in infants, often linked to Kasabach-Merritt phenomenon. Early diagnosis and multimodal treatment led to complete remission in a 3-month-old infant.
Area of Science:
- Pediatric Oncology
- Vascular Anomalies
- Pediatric Surgery
Background:
- Kaposiform hemangioendothelioma (KHE) is a rare, aggressive vascular tumor predominantly affecting infants.
- KHE is frequently complicated by Kasabach-Merritt phenomenon (KMP), a life-threatening coagulopathy.
- Optimal management strategies for KHE remain challenging due to its rarity and complexity.
Purpose of the Study:
- To present a case of successful diagnosis and treatment of infantile Kaposiform hemangioendothelioma.
- To highlight the importance of early diagnosis and multimodal therapy in managing KHE.
- To demonstrate a favorable outcome in a complex pediatric vascular tumor case.
Main Methods:
- Diagnostic workup included Magnetic Resonance Imaging (MRI) and tissue biopsy.
- Treatment involved a multimodal approach: surgical excision, corticosteroids, propranolol, vincristine, and supportive care for coagulopathy.
- Clinical monitoring and follow-up assessments were conducted.
Main Results:
- A 3-month-old female infant was diagnosed with Kaposiform hemangioendothelioma.
- The infant received successful treatment combining surgical and medical interventions.
- The patient achieved complete remission with no evidence of recurrence.
Conclusions:
- Kaposiform hemangioendothelioma, despite its aggressive nature, can be effectively managed with early diagnosis and a comprehensive treatment plan.
- Multimodal therapy, including surgical and medical options, is crucial for achieving favorable outcomes in KHE.
- This case underscores the potential for complete remission in infants with KHE and associated Kasabach-Merritt phenomenon.
