Department of Radiology, Queen Mary Hospital, 102 Pokfulam Road, Hong Kong, Hong Kong.
Department of Radiology, Hong Kong Children's Hospital, Hong Kong, Hong Kong.
Skeletal Radiol. 2023 Apr;52(4):783-790. doi: 10.1007/s00256-022-04152-z. Epub 2022 Aug 10.
Kaposiform haemangioendothelioma (KHE) is a rare childhood disease classified by the International Society for the Study of Vascular Anomalies (ISSVA) as a locally aggressive vascular tumor. It has been reported to affect any site, with a predilection for the extremities and trunk. Although it typically manifests as an enlarging cutaneous or soft tissue lesion, less than 10% of cases have no skin involvement, with the retroperitoneum being the most frequently involved extracutaneous site. Approximately twenty cases of KHE with bony involvement have been reported in the literature to date, with only five of those cases involving the spine specifically.We present a, rare case of KHE who presented with progressive fixed hyperlordotic deformity, multiple non-specific spinal lesions, and abnormal blood tests, posing a clinical and radiological diagnostic challenge. Additionally, we conducted a thorough review of the literature to compare and contrast the various multimodality imaging manifestations of KHE involving the spine.
卡波西样血管内皮细胞瘤(KHE)是一种罕见的儿童疾病,国际脉管性疾病研究学会(ISSVA)将其归类为具有局部侵袭性的血管肿瘤。它可发生于任何部位,好发于四肢和躯干。尽管其典型表现为增大的皮肤或软组织病变,但小于 10%的病例无皮肤受累,腹膜后是最常累及的皮肤外部位。迄今为止,文献中已有约 20 例 KHE 伴骨受累的报道,其中仅有 5 例为脊柱受累。我们报告了一例罕见的 KHE 病例,该患者表现为进行性固定性过度脊柱前凸畸形、多个非特异性脊柱病变和异常的血液检查,这对临床和影像学诊断构成了挑战。此外,我们还对文献进行了全面回顾,以比较和对比涉及脊柱的 KHE 的各种多模态影像学表现。