Multifocal Kaposiform Hemangioendothelioma Successfully Treated With Sirolimus Monotherapy

Area of Science:

• Vascular Tumors
• Pediatric Oncology
• Dermatology

Background:

• Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm predominantly affecting infants.
• KHE can present multifocally and may be associated with Kasabach-Merritt phenomenon (KMP), a serious coagulopathy.
• Early and accurate diagnosis is crucial for effective management.

Observation:

• A case of multifocal KHE on an infant's leg, initially misdiagnosed as a reticulate port wine birthmark.
• Diagnostic confirmation was achieved through skin biopsy and advanced imaging.
• The patient developed Kasabach-Merritt phenomenon (KMP), indicating disease complexity.

Findings:

• Multifocal KHE on the leg was confirmed via histopathology and imaging.
• Sirolimus monotherapy was initiated due to the presence of KMP.
• Significant clinical improvement was observed following sirolimus treatment.

Implications:

• This case highlights the importance of considering KHE in infants with atypical vascular lesions.
• Sirolimus monotherapy demonstrates efficacy in managing complex KHE cases with KMP.
• Further research into optimal KHE and KMP treatment strategies is warranted.