Pilomyxoid astrocytomas (PMA) are new class of Pilocytic Astrocytoma (PA.), which typically have their origin in hypothalamus and Chiasmatic region. There are very few case reports of PMAs arising from cerebellum. Their imaging features are similar to PA but they behave more aggressively than PA. To increase awareness of PMA within the neurosurgical community, the authors reviewed a case of 11-year-old male child who presented with truncal and cerebellar ataxia and vomiting and right cerebellar tumor diagnosed as PA radiologically but PMA on histopathology examination. These findings indicate that PMA may be a unique entity that is distinct from PA, or it may be an unusual variant.Key words: -Pilocytic astrocytoma • pilomyxoid astrocytoma • diagnosis • cerebellar DOI: http://dx.doi.org/10.4314/ecajs.v21i3.22
IntroductionPilocytic astrocytomas are generally benign, typically showing 20-year survival rates of 70 to 80%, even when only a subtotal resection is achieved. 1 These highly treatable and potentially curable pediatric tumors are considered Grade I neoplasms according to the World Health Organization tumor classification system. 2, 3 Prior to its recognition, PMA was grouped with PA, because the two display similar histological features.In 1999, Tihan and colleagues provided the sentinel description of PMAs. 1 Because of their similar histological and radiological features, 2 PMAs were classified as PAs prior to acceptance of this publication. . Earlier reports pointed out unusual features of some pediatric astrocytomas, particularly those within the hypothalamic/chiasmatic region, but did not specifically use a term to distinguish them. 4,5,9 In contrast to PAs, PMAs demonstrate a more aggressive clinical course 8-9 and appear to be associated with a higher incidence of leptomeningeal spread. 9 Pilomyxoid astrocytomas are considered to be WHO Grade II neoplasms, and most often arise from the hypothalamic/chiasmatic region. However, PMAs sometimes originate from the posterior fossa. 4 whereas hypothalamic PMAs have been well described in the literature, there is a relative paucity of information about PMAs that arise from the cerebellum. Although limited clinical experience makes it difficult to generate conclusive prognostic data regarding this recently described pediatric tumor, PMA has been shown to behave more aggressively than PA . 11 Here we report a case of cerebellar PMA in a 11 year old male child and discuss literature review
Case reportThis was an 11 year old male patient who was relatively in a good state of health prior to his presentation to neurosurgical referral clinics at which time he presented with global headache,projectile vomiting of ingested matter of one moth duration, these symptoms were associated with difficulty of maintaining balance, and blurring of vision.Physical examination revealed papilledema with positive Romberg's test.Abnormal rapid alternating movements, and tandemwalk were indicative of cerebellar ataxia and truncal ataxia. Brain CT scan with contrast was done, With di...