Multi focal Pilocytic Astrocytomas With Ependymal Differentiation in the Bilateral Medial Temporal Lobes-Case Report-

Abstract

A 7-year-old boy presented with headache, visual disturbance, and psychomotor seizures persisting for 7 months. He had mild hemiparesis and homonymous hemianopia on the left. Neuroimaging showed bilateral temporal lobe masses with calcification and cysts. The right temporal mass was subtotally resected. The histological diagnosis was pilocytic astrocytoma with ependymal differentiation and a MIB-1 staining index of up to 8.0%. Postoperatively his hemiparesis and psychomotor seizures disappeared. Adjuvant chemotherapy consisting of carboplatin and vincristine was given followed by radiotherapy. Neuroimaging showed that the bilateral tumors had disappeared and showed no recurrence for 29 months after the diagnosis. Pilocytic astrocytoma usually presents as a solitary mass in the cerebellum or optic pathway with low proliferative activity, but should be included in the differential diagnosis of multifocal tumors arising in the bilateral temporal lobes. Ependymal differentiation with extremely high proliferative activity might be related to this unusual clinical presentation. Intensive treatment is recommended for patients with such specific neuroimaging and histological features.

Diagnosis and Management of Multifocal Gliomas

“…In such cases, early detection of multifocal juvenile pilocytic astrocytoma by MRI may change treatment strategies and improve prognosis [24]. Thus, even though juvenile pilocytic astrocytoma usually presents as a solitary mass in the cerebellum or optic pathway with low proliferative activity, it occasionally presents as multifocal tumor with more aggressive behavior [25,26]. In a study of 90 patients with either primary or recurrent pilocytic astrocytoma, 11 patients developed multicentric spread.…”

Neuropathological spectrum of pilocytic astrocytoma-an Indian series of 120 cases

“…Gilles et al 14 noted parenchymal calcification more among older children, while Philipson et al 30 observed calcification and ossification in a case of PA involving spinal nerve root in a 39-year-old woman. Dierssen et al 11 also described extensive calcification in a case of pontine PA. Maruyama et al 28 and Lee et al 24 reviewed the CT and MR characteristics of juvenile pilocytic astrocytomas and noted tumor calcification in occasional cases.…”

“…Other features that are variably present in PA include sclerotic vessels, vascular proliferation, calcification 7,11,14,24,27,28,30 and chronic inflammation, 7,14,27 while nuclear pleomorphism, mitosis and necrosis are rare. Owing to the differences in prognosis and therapeutic options, it is vital to distinguish PA from diffuse astrocytoma, and in the presence of mitosis / necrosis from other high-grade neoplasm.…”