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Posterior Fossa Neoplasm and PHACES Syndrome: A Case Report

Wallen, Katharine E. MD*; Hadar, Eldad J. MD; Perry, Victor MD; Bouldin, Thomas W. MD; Loehr, James MD§; Blatt, Julie MD*

Journal of Pediatric Hematology/Oncology: March 2009 - Volume 31 - Issue 3 - pp 203-205
doi: 10.1097/MPH.0b013e3181983b15
Clinical and Laboratory Observations

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.

*Divisions of Hematology Oncology

Neurosurgery

§Cardiology

Departments of Pediatrics and Pathology/Laboratory Medicine, The University of North Carolina, Chapel Hill, NC

No financial support was received for the production of this case report.

Reprints: Katharine E. Wallen, MD, Division of Pediatric Hematology Oncology, Room 1106, 170 Manning Drive, University of North Carolina, Chapel Hill, NC 27599-7236 (e-mail: kwallen@unch.unc.edu).

Received for publication August 8, 2008; accepted November 18, 2008

© 2009 Lippincott Williams & Wilkins, Inc.