Pilomyxoid astrocytomas (PMA) is a particular form of glial tumors distinct from pilocytic
astrocytomas (PA). On the last 2016 WHO classification for CNS tumours, no definite
grade assignment was proposed for these lesions. They may be more aggressive with
a different clinical course compared to PA due to their greater propensity for local
recurrence and cerebrospinal dissemination. Most cases arise from the hypothalamic
region. Only few studies reported cerebellar localization of the lesion. We report
3 pediatric cases treated for pediatric PMA of the posterior fossa. Clinical, radiological,
and prognostic features were reviewed. The age of our patients was between 1 and 9
years old. Signs of intracranial hypertension were found in all patients. One of them
presented an increased head circumference and the 2 others had a cerebellar syndrome.
Brain CT-scan and MRI displayed a large wellcircumscribed intra-axial solid and cystic
posterior fossa tumor. Total surgical resection was performed for all tumors. After
a 2 years follow up, no signs of recurrence were noticed. In the literature, PMA been
reported with overwhelming majority in children aged between 2 months and 4 years.
Despite of many pathological similarities with PAs, PMAs have some specific features
in histology, leading to their identification as independent type of glioma. Radiological
differential diagnosis between PMAs and Pas can be made using arterial spin labeling
imaging, which shows low perfusion parameters in PAs. Clinical and radiological follow
up are mandatory do to different natural history and higher rates of local recurrence
of this tumor compared to PA. Prognosis is favorable when complete surgical exeresis
is possible.
Key-words:
Astrocytoma - neurosurgery - pediatrics