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January 13, 2022

Kaposiform Hemangioendothelioma of Internal Auditory Canal Presenting in 4-Week-Old Newborn

Author Affiliations
  • 1New York Medical College, Valhalla, New York
  • 2Department of Otolaryngology, Westchester Medical Center, Valhalla, New York
  • 3Department of Neurosurgery, Westchester Medical Center, Valhalla, New York
  • 4Department of Pathology, Westchester Medical Center, Valhalla, New York
JAMA Otolaryngol Head Neck Surg. 2022;148(3):282-284. doi:10.1001/jamaoto.2021.4010

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor. It is classified by the World Health Organization (WHO) as intermediate malignant.1 Some cases have been associated with Kasabach-Merritt syndrome (KMS), a phenomenon defined by thrombocytopenia and coagulopathy, increasing the risk for life-threatening hemorrhage.1,2 Typically, KHE is found in pediatric patients.1 The most common locations for presentation are the extremities, trunk, and retroperitoneum. A smaller number of cases have been reported in the head and neck.3 To our knowledge, there has been only 1 previous report of KHE in the internal auditory canal.4 We present what we believe to be the youngest patient with an internal auditory canal KHE.

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