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Case Report: Kaposiform Hemangioendothelioma With Spinal Involvement
Tong Qiu1, Kaiying Yang1, Shiyi Dai1
1Division of Oncology, Department of Pediatric Surgery, West China Hospital of Sichuan University, Chengdu, China.
Frontiers in Pediatrics
|April 29, 2021
Summary
Kaponiform hemangioendothelioma (KHE) is a rare vascular tumor. Spinal KHE presents with varied symptoms, but sirolimus treatment shows promising outcomes in patients.
Area of Science:
- Pediatric Oncology
- Vascular Malformations
- Spinal Tumors
Background:
- Kaponiform hemangioendothelioma (KHE) is a rare, locally invasive vascular tumor primarily affecting infants and adolescents.
- Spinal involvement in KHE is exceptionally rare, presenting diagnostic and therapeutic challenges.
Observation:
- This study reviewed seven pediatric patients with spinal KHE, noting symptoms like pain, decreased range of motion, scoliosis, claudication, and soft tissue masses.
- Five patients (71.4%) exhibited the Kasabach-Merritt phenomenon (KMP), a serious complication characterized by thrombocytopenia.
Findings:
- All seven patients were treated with sirolimus, resulting in lesion regression and/or normalization of hematologic parameters.
- Early recognition of KHE's variable symptoms is crucial for timely intervention.
Implications:
- Spinal KHE requires a multidisciplinary approach for optimal management.
- Sirolimus demonstrates efficacy in improving outcomes for KHE with spinal involvement, offering a new therapeutic avenue.
