Overview
Retiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor classified by the International Society for the Study of Vascular Anomalies (ISSVA). It predominantly affects children, often presenting as cutaneous or soft tissue lesions, with less than 10% lacking skin involvement; bony involvement, particularly in the spine, is exceedingly rare 1.Diagnosis
Clinical Presentation: Enlarging cutaneous or soft tissue lesions, potentially with bony involvement 1.
Imaging: Multimodality imaging (MRI, CT) essential for assessing extent and involvement of spinal lesions 1.
Laboratory Findings: Abnormal blood tests may indicate consumptive coagulopathy or Kasabach-Merritt Syndrome 1.
Histopathology: Definitive diagnosis through biopsy showing characteristic retiform or anastomosing vascular channels 1.Management
First-Line Treatment: Surgical excision when feasible, especially for localized lesions 1.
Adjunctive Therapies:
- Radiation Therapy: Considered for unresectable or recurrent disease 1.
- Systemic Therapy: No specific drug classes or doses mentioned in the abstract 1.
Supportive Care: Management of coagulopathy and symptoms related to Kasabach-Merritt Syndrome 1.Special Populations
Pediatrics: KHE predominantly affects children, requiring careful monitoring and multidisciplinary care 1.
Comorbidities: Cases involving bony deformities or Kasabach-Merritt Syndrome necessitate specialized management 1.Key Recommendations
Multimodality imaging is crucial for diagnosing and assessing the extent of spinal involvement in KHE (Evidence: Moderate 1).
Surgical excision should be considered for localized spinal lesions to achieve local control (Evidence: Expert opinion 1).
Monitor for and manage coagulopathy associated with Kasabach-Merritt Syndrome in affected patients (Evidence: Moderate 1).References
1 Chin LHQ, Fung KKF, Chan JPK, Kan ANC, Yuen MK. Kaposiform haemangioendothelioma of the spine associated with fixed hyperlordotic deformity and Kasabach-Merritt Syndrome: a case report and literature review. Skeletal radiology 2023. link