Department of Diagnostic and Interventional Radiology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8, Canada.
Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada.
Pediatr Radiol. 2024 Oct;54(11):1873-1883. doi: 10.1007/s00247-024-06041-0. Epub 2024 Sep 3.
Angiosarcomas are rare malignant vascular tumors and there is scarcity of data on their imaging features.
To review and illustrate the imaging, clinical, and pathologic features of angiosarcoma in children.
A list of pathologically proven angiosarcoma seen between Nov 1992 and Jan 2023 was obtained from a pathology database and picture archiving and communication system. Those with pre-treatment imaging available on our PACS were included in the study. Imaging studies were reviewed by two readers in consensus.
A total of six children (two males and four females; median age of 8.8 years; range 2.9 years to 15.5 years) had angiosarcoma during the study period. Organ of origin included breast (n = 2), liver (n = 2), spleen (n = 1), and paranasal sinuses (n = 1). The patient with splenic angiosarcoma had Li-Fraumeni syndrome. Five patients had a single lesion while one had multifocal lesions. The tumors were large with a median diameter of 12.9 cm (range 2.7 cm to 24 cm). Most tumors were heterogeneous on T2-weighted imaging with hemorrhage and necrosis and showed heterogeneous enhancement. Three had well-defined borders and three had infiltrative borders. None of the tumors showed calcifications. Two tumors in the liver showed gradual non-centripetal progressive diffuse enhancement on dynamic imaging. One patient had metastases at presentation and four patients subsequently developed metastases on follow-up. Five patients underwent surgical resection and chemotherapy; one patient with a liver lesion underwent arterial embolization followed by liver transplant. Three patients died at the last follow-up.
The imaging features of angiosarcomas are nonspecific, but the tumors are large heterogeneously enhancing masses with hemorrhage and necrosis. Hepatic angiosarcomas may show non-centripetal progressive and heterogeneous enhancement on dynamic imaging.
血管肉瘤是一种罕见的恶性血管肿瘤，其影像学特征数据较少。
回顾和阐述儿童血管肉瘤的影像学、临床和病理特征。
从病理数据库和影像归档与通信系统中获得 1992 年 11 月至 2023 年 1 月期间经病理证实的血管肉瘤病例列表。将在我们的影像归档与通信系统中有治疗前影像学资料的病例纳入研究。由两名读者共同对影像学研究进行审查。
在研究期间，共有 6 名儿童（2 名男性和 4 名女性；中位年龄 8.8 岁；范围 2.9 岁至 15.5 岁）患有血管肉瘤。原发部位包括乳房（n=2）、肝脏（n=2）、脾脏（n=1）和鼻旁窦（n=1）。患有脾血管肉瘤的患者患有 Li-Fraumeni 综合征。5 名患者有单个病灶，1 名患者有多个病灶。肿瘤较大，中位直径为 12.9cm（范围 2.7cm 至 24cm）。大多数肿瘤在 T2 加权成像上呈不均匀性，伴有出血和坏死，并呈不均匀增强。3 个肿瘤边界清楚，3 个肿瘤边界浸润。无肿瘤钙化。肝脏的 2 个肿瘤在动态成像上表现为逐渐的非向心性弥漫性增强。1 名患者在就诊时出现转移，4 名患者在随访中出现转移。5 名患者接受了手术切除和化疗；1 名肝脏病变患者接受了动脉栓塞后肝移植。3 名患者在最后一次随访时死亡。
血管肉瘤的影像学特征不具有特异性，但肿瘤是大的不均匀增强肿块，伴有出血和坏死。肝血管肉瘤在动态成像上可能表现为非向心性进行性和不均匀性增强。
