Kaposiform Hemangioendothelioma in the Thoracic Spine: A Case Report and Review of the Literature

Abstract

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.

Clinical and imaging features of Kaposiform hemangioendothelioma in infants

“…KHE, first described by Zukerberg et al, in 1993, is a rare vascular tumor that occurs most frequently in the deep soft tissues of the extremities [ 4 , 5 ]. The overall incidence is low, and KHE predominantly occurs in infants and children, with most of the lesions located in the soft tissues of the extremities and trunk and a few diffuse lesions involving the mediastinum, retroperitoneal tissues, intracranial tissues, and spine [ 3 , [6] , [7] , [8] ]. In their study of 165 patients, Fernandez et al showed that most patients were children under 12 years of age and that 60 patients had lesions located in the extremities, with the remaining patients showing involvement in multiple sites [ 9 ].…”