Premium
Pilomyxoid astrocytoma in a patient with neurofibromatosis
Author(s) -
Khanani Muhammad Faisal,
Hawkins Cynthia,
Shroff Manohar,
Dirks Peter,
Capra Michael,
Burger P.C.,
Bouffet Eric
Publication year - 2006
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.20391
Subject(s) - medicine , neurofibromatosis , astrocytoma , dermatology , pathology , glioblastoma , cancer research
Pilomyxoid astrocytoma (PMA), a recently described variant of low‐grade astrocytoma is associated with a high rate of recurrence and a propensity for CSF seeding. While cases of PMA have been reported in infants and young children, there has been no report of PMA in patients with neurofibromatosis. The first reportable case of PMA occurring in a child with neurofibromatosis type 1 (NF1) is described. Following presentation with obstructive hydrocephalus, the patient underwent a partial resection of a third ventricular tumor. Histology confirmed the typical features of PMA. The patient demonstrated a partial response to chemotherapy. The authors review the literature on PMA and discuss the specific issues associated with this diagnosis in the context of a child with neurofibromatosis. © 2005 Wiley‐Liss, Inc.