Posterior fossa neoplasm and PHACES syndrome: a case report.
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.
Wallen
Katharine E
KE
Division of Pediatric Hematology Oncology, The University of North Carolina, Chapel Hill, NC 27599-7236, USA. kwallen@unch.unc.edu
Hadar
Eldad J
EJ
Perry
Victor
V
Bouldin
Thomas W
TW
Loehr
James
J
Blatt
Julie
J
D000015:Abnormalities, Multiple / Q000473:pathology / Q000503:physiopathology; D001254:Astrocytoma / Q000209:etiology* / Q000473:pathology; D001921:Brain / Q000002:abnormalities* / Q000473:pathology; D002675:Child, Preschool; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000150:complications; D006391:Hemangioma / Q000150:complications* / Q000151:congenital; D006801:Humans; D015192:Infratentorial Neoplasms / Q000209:etiology* / Q000473:pathology; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D013577:Syndrome