Kaposiform Hemangioendothelioma Arising From the Maxillary Sinus: A Case Report

• Published in: The Kaohsiung journal of medical sciences Vol. 26; no. 3; pp. 154 - 157
• Main Authors: Lee, Chin-Ho, Jaw, Twei-Shiun, Yang, Sheau-Fang, Wu, Ding-Kwo
• Format: Journal Article
• Language: English
• Published: China (Republic : 1949- ) Elsevier B.V 01.03.2010; Wiley
• Subjects: Female; head and neck; Hemangioendothelioma - diagnostic imaging; Hemangioendothelioma - pathology; Humans; Infant; Internal Medicine; kaposiform hemangioendothelioma; Kasabach-Merritt phenomenon; Magnetic Resonance Imaging; Maxillary Sinus - diagnostic imaging; Maxillary Sinus - pathology; Tomography, X-Ray Computed; Kasabach-Merritt phenomenon; head and neck; kaposiform hemangioendothelioma
• ISSN: 1607-551X; 2410-8650
• DOI: 10.1016/S1607-551X(10)70023-1

Kaposiform hemangioendothelioma is a very infrequent, locally aggressive vascular neoplasm, characterized by fascicular spindle cell proliferation. It occurs almost exclusively in infants and adolescents, and is often associated with Kasabach-Merritt phenomenon. The tumor is predominantly located subcutaneously or in the deep soft tissue of the extremities and trunk, peritoneum, or retroperitoneum. However, this tumor can sometimes be located on the head and neck. We report a case of kaposiform hemangioendothelioma of the maxillary sinus in a 4-month-old female infant presenting with cheek swelling, thrombocytopenia, anemia, and disseminated intravascular coagulation. Sinus computed tomography presented an enhancing, bone-destructing tumor. Magnetic resonance images showed an uncommon appearance as mostly low signal intensity on T2-weighted images.