Background: Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor of childhood. It has been most commonly reported in the trunk, retroperitoneum, and extremities, but cases involving the head and neck have been described. While thought to have limited metastatic potential, it is associated with Kasabach-Merritt syndrome (KMS), a consumptive coagulopathy and profound thrombocytopenia that is associated with significant mortality. Twelve cases of KHE with bony involvement have previously been reported. Of these, only 2 involved the spine, and both were managed with medical therapy alone.
Case presentation: We report the first case of spinal KHE causing spinal cord compression managed with surgical excision presented in the literature to date.
Conclusions: There are a number of treatment modalities outlined in the literature. The lack of a consensus is due to the varying methods of presentation, difficulty in diagnosis, and disease rarity. Our case highlights the potential speed of local growth of this tumor, meaning that excision may be a technical challenge, especially when there is epidural extension. Treating clinicians should be aware of the risk of progression beyond the initial tumor boundaries to involve new vertebrae.
Keywords: Kasabach-Merritt syndrome (KMS); kaposiform hemangioendothelioma (KHE); malignant cord compression; vascular tumor.
©International Society for the Advancement of Spine Surgery 2020.