Grassia Kalee L, Peterman Caitlin M, Iacobas Ionela, Margolin Judith F, Bien Ewa, Padhye Bhavna, Meyers Rebecka L, Adams Denise M
University of Cincinnati College of Medicine, Cincinnati, Ohio.
Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Pediatr Blood Cancer. 2017 Nov;64(11). doi: 10.1002/pbc.26627. Epub 2017 May 18.
Hepatic angiosarcoma is a rare, aggressive, malignant neoplasm with fewer than 50 cases reported in children. Prognosis is poor, with a minority surviving beyond 2 years after diagnosis. We report eight cases of pediatric hepatic angiosarcoma, diagnosed at a mean age of 3 years. Seven were initially diagnosed with an infantile hepatic hemangioendothelioma (IHHE) or hemangioma and the eighth with a "vascular tumor." Two patients, who received liver transplant, survived. We suggest hepatic hemangiomas can rarely transform into angiosarcomas and a subset of IHHEs (Type II) are actually a low-grade form of angiosarcoma rather than a benign lesion.
肝血管肉瘤是一种罕见的、侵袭性恶性肿瘤,儿童病例报告少于50例。预后较差,少数患者在诊断后存活超过2年。我们报告8例儿童肝血管肉瘤,诊断时平均年龄为3岁。7例最初被诊断为婴儿肝血管内皮瘤(IHHE)或血管瘤,第8例被诊断为“血管肿瘤”。2例接受肝移植的患者存活。我们认为肝血管瘤很少能转变为血管肉瘤,一部分IHHE(II型)实际上是低级别血管肉瘤而非良性病变。