Kaposiform hemangioendothelioma of the bone in children and adolescents

• Published in: Pediatric blood & cancer Vol. 69; no. 1; pp. e29392 - n/a
• Main Authors: Kuo, Christopher, Warren, Mikako, Malvar, Jemily, Miller, Joseph M., Shah, Rachana, Navid, Fariba, Luu, Minnelly, Anselmo, Dean M., Mascarenhas, Leo
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01-01-2022
• Subjects: Adolescent; Bone; Case reports; Child; Child, Preschool; Children; Hemangioendothelioma - diagnostic imaging; Hematology; Humans; Kaposiform hemangioendothelioma; Kasabach-Merritt Syndrome; Kassabach‐Merritt phenomenon; Oncology; Pediatrics; Sarcoma; Sarcoma, Kaposi - diagnosis; sirolimus; Tumors; Vascular Neoplasms; Bone; Kassabach-Merritt phenomenon; Kaposiform hemangioendothelioma; sirolimus
• ISSN: 1545-5009; 1545-5017
• DOI: 10.1002/pbc.29392

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor that mainly occurs during infancy or early childhood. Approximately 70% of cases are complicated by Kasabach‐Merritt phenomenon. Although osseous extension of the primary lesion is relatively common, primary bone involvement by KHE is rare. Given the paucity of literature on primary KHE of the bone, we report a case series of primary KHE of the bone treated at our institution and describe the clinical presentation, radiologic and pathologic findings, management and outcomes.