Wallen Katharine E, Hadar Eldad J, Perry Victor, Bouldin Thomas W, Loehr James, Blatt Julie
Division of Pediatric Hematology Oncology, The University of North Carolina, Chapel Hill, NC 27599-7236, USA.
J Pediatr Hematol Oncol. 2009 Mar;31(3):203-5. doi: 10.1097/MPH.0b013e3181983b15.
A 4-year-old girl with PHACES syndrome (posterior fossa brain malformations, hemangiomas, arterial anomalies, cardiac anomalies/coarctation of the aorta, eye abnormalities, and sternal clefting/supraumbilical raphe) developed a cerebellar pilocytic astrocytoma 18 months after resolution of her neck, ear, and thoracic hemangiomas. Because cutaneous hemangiomas may have involuted by the time a patient is diagnosed with a central nervous system neoplasm, it seems possible that in other such patients the association may have gone unrecognized. Cerebellar pilocytic astrocytoma may be a rare manifestation of the posterior fossa malformations of PHACES.
一名患有PHACES综合征(后颅窝脑畸形、血管瘤、动脉异常、心脏异常/主动脉缩窄、眼部异常以及胸骨裂/脐上缝)的4岁女孩,在其颈部、耳部和胸部血管瘤消退18个月后,患上了小脑毛细胞型星形细胞瘤。由于在患者被诊断出患有中枢神经系统肿瘤时,皮肤血管瘤可能已经消退,所以在其他此类患者中,这种关联可能未被识别。小脑毛细胞型星形细胞瘤可能是PHACES后颅窝畸形的一种罕见表现。