Kaposiform Hemangioendothelioma Arising from the Maxillary Sinus: A Case Report

卡波西樣血管內皮細胞瘤是一種非常罕見的，具局部侵犯性的血管腫瘤，其特徵為束狀梭形細胞增生。它幾乎全部發生在嬰幼兒和青少年，經常合併有卡－梅綜合徵。腫瘤主要位於皮下或深部軟組織的四肢和軀幹，腹腔或後腹膜腔。然而，頭部和頸部是例外。我們報告一例上頜竇的卡波西樣血管內皮細胞瘤在4個月大的女嬰呈現臉頰腫脹，血小板減少，貧血，瀰漫性血管內凝血。鼻竇電腦斷層呈現一個有顯影的骨破壞腫瘤。而磁共振成像顯示少見地在T2加權像大部分以低信號強度作為表現。

關鍵字

頭部和頸部；卡波西樣血管內皮細胞瘤；卡－梅綜合徵；上頜竇

並列摘要

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.

並列關鍵字

head and neck ； kaposiform hemangioendothelioma ； Kasabach-Merritt phenomenon ； maxillary sinus

國際替代計量

Kaposiform Hemangioendothelioma Arising from the Maxillary Sinus: A Case Report

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