Ryu Young Jin, Choi Young Hun, Cheon Jung-Eun, Kim Woo Sun, Kim In-One, Park Ji Eun, Kim Yu Jin
Department of Radiology, Seoul National University Hospital, Seoul, Republic of Korea; Department of Radiology, Seoul National University College of Medicine, Seoul, Republic of Korea.
Department of Radiology, Seoul National University Hospital, Seoul, Republic of Korea; Department of Radiology, Seoul National University College of Medicine, Seoul, Republic of Korea.
Eur J Radiol. 2017 Jan;86:198-205. doi: 10.1016/j.ejrad.2016.11.015. Epub 2016 Nov 10.
Kaposiform hemangioendothelioma (KHE) is a rare, aggressive vascular tumor that typically occurs during infancy or early childhood. Though several case reports have discussed the imaging findings of KHE, larger comprehensive studies are lacking. The purpose of this study was to evaluate the imaging findings of KHE in children.
A total of twelve cases of pathologically proven KHE were collected by searching our institution's pathology database for children diagnosed between January 2004 and April 2016 (6 male, 6 female; median age: 3 months; age range 7days - 18 years). CT (n=7) and MRI (n=9) findings were retrospectively evaluated. The location, morphology, enhancement, and growth pattern were analyzed.
KHEs involved various locations: superficial or deep soft tissue of the extremities (n=4); abdomen (n=3; 2 cases, pancreas; 1 case, small bowel), neck and mediastinum (n=1); chest wall, diaphragm, and pericardium (n=1); abdominal wall (n=1); and head (IAC, CP angle) (n=2). Eight of 10 cases (80%) with CT/MR findings were locally invasive and involved two or more adjacent organs. Three cases presented as well-defined solid masses, and seven were associated with infiltrative lesions of the surrounding areas with (n=4) or without definite solid regions (n=3). In nine patients with MRI, all KHEs were heterogeneous and hyperintense to muscles on T2-weighted images (T2WI), while four KHEs consisted of some regions that were nearly isointense to muscle. Eight of the 10 cases with imaging exhibited heterogeneous intense enhancement, while only one demonstrated mild enhancement. Signal voids (n=2), engorged vessels (n=1), calcification (n=3), hemorrhage (n=1), or bone changes (n=4) were infrequently observed. Four patients (33%) had Kasabach-Merritt phenomenon, and recurrence was observed in two cases.
KHEs occurred in various locations, affected mostly infants, and generally exhibited intense heterogeneous enhancement. In more than half of the included cases, KHEs were highly infiltrative and locally invasive with ill-defined margins. Awareness of these features should prompt radiologists to include KHE in the differential diagnosis for pediatric masses.
卡波西样血管内皮瘤（KHE）是一种罕见的侵袭性血管肿瘤，通常发生于婴儿期或儿童早期。尽管有几例病例报告讨论了KHE的影像学表现，但缺乏更大规模的综合研究。本研究的目的是评估儿童KHE的影像学表现。
通过检索我院2004年1月至2016年4月诊断的儿童病理数据库，共收集了12例经病理证实的KHE病例（男6例，女6例；中位年龄：3个月；年龄范围7天至18岁）。对CT（n=7）和MRI（n=9）表现进行回顾性评估。分析其位置、形态、强化及生长方式。
KHE累及多个部位：四肢浅表或深部软组织（n=4）；腹部（n=3，2例累及胰腺，1例累及小肠）、颈部及纵隔（n=1）；胸壁、膈肌及心包（n=1）；腹壁（n=1）；头部（内听道、小脑脑桥角）（n=2）。10例有CT/MR表现的病例中，8例（80%）呈局部侵袭性，累及两个或更多相邻器官。3例表现为边界清晰的实性肿块，7例伴有周围区域的浸润性病变，其中4例有明确实性区域，3例无明确实性区域。9例行MRI检查的患者中，所有KHE在T2加权像（T2WI）上均呈不均匀高信号，高于肌肉，4例KHE部分区域信号与肌肉接近等信号。10例有影像学表现的病例中，8例呈不均匀明显强化，仅1例呈轻度强化。较少观察到信号缺失（n=2）、血管增粗（n=1）、钙化（n=3）、出血（n=1）或骨质改变（n=4）。四名患者（33%）出现卡萨巴赫-梅里特现象，2例出现复发。
KHE发生于多个部位，主要影响婴儿，通常表现为明显的不均匀强化。在超过半数纳入病例中，KHE具有高度浸润性和局部侵袭性，边界不清。了解这些特征应促使放射科医生在儿科肿块的鉴别诊断中考虑KHE。
