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大腿部卡波西型血管内皮瘤:病例报告

Kaposiform hemangioendothelioma of the thigh: A case report.

作者信息

Morgado Margarida, Alves Filipe Barros, Pedrosa Sofia, Garcia Maria, Dias Sílvia Costa, Madureira António J

机构信息

Department of Radiology, Centro Hospitalar Universitário de São João, Alameda Prof. Hernâni Monteiro, Porto, Portugal.

Department of Pathology, Centro Hospitalar Universitário de São João, Alameda Prof. Hernâni Monteiro, Porto, Portugal.

出版信息

Radiol Case Rep. 2023 Jun 26;18(9):3152-3156. doi: 10.1016/j.radcr.2023.05.052. eCollection 2023 Sep.

Abstract

Kaposiform hemangioendothelioma is a rare, locally aggressive or borderline vascular tumor that typically affects infants. It presents as a purpuric cutaneous lesion and may be associated with life-threatening coagulation disorders, such as the Kasabach-Merritt phenomenon. The differential diagnosis can be challenging based on clinical presentation alone. Imaging plays a crucial role in the diagnostic workup, particularly magnetic resonance imaging. We present a case report of a 4-month-old patient with an enlarging vinous cutaneous mass on the thigh and coagulation abnormalities. Magnetic resonance imaging revealed a large, infiltrative, soft-tissue lesion with poorly defined margins and heterogeneous enhancement, that involved all muscle compartments of the thigh and was associated with lymphedema, stranding of the subcutaneous fat and cutaneous thickening. These findings were consistent with kaposiform hemangioendothelioma of the thigh and the diagnosis was confirmed by histopathological characterization.

摘要

卡波西样血管内皮瘤是一种罕见的、具有局部侵袭性或介于良恶性之间的血管肿瘤,通常影响婴儿。它表现为紫癜性皮肤病变,可能与危及生命的凝血障碍有关,如卡萨巴赫-梅里特现象。仅根据临床表现进行鉴别诊断可能具有挑战性。影像学检查在诊断工作中起着关键作用,尤其是磁共振成像。我们报告一例4个月大的患者,其大腿部有一个不断增大的静脉性皮肤肿物,并伴有凝血异常。磁共振成像显示一个大的、浸润性的软组织病变,边界不清,强化不均匀,累及大腿的所有肌肉间隙,并伴有淋巴水肿、皮下脂肪条索状改变和皮肤增厚。这些表现符合大腿部卡波西样血管内皮瘤,组织病理学特征证实了诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b64e/10318450/cb8194006957/gr1.jpg

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