Rare vermian pilocytic astrocytoma with recurrent spontaneous hemorrhage in the elderly: A case report and review of literature

Abstract

Background: Pilocytic astrocytoma (PA) is a benign glial tumor predominately seen in pediatrics and early adolescence with associated overall good outcomes. Very few cases of elderly PA have been reported in the literature, and they are known to display unique anatomic, histologic, and genetic peculiarities distinct from pediatric disease. We report a rare case of vermian PA in an octogenarian with recurrent spontaneous intratumoral hemorrhage as a presenting symptom. Furthermore, a review of the literature on the peculiarities of PA in the elderly will be discussed.

Case description: An 81-year-old woman presented with features suggestive of repeated posterior fossa hemorrhages characterized by headaches, diplopia, and alteration in sensorium occurring about 5 months apart. Brain neuroimaging showed a cerebellar vermian tumor with features suggestive of repeated intratumoral bleeding. She had an initial ventriculoperitoneal shunting for acute hydrocephalus and subsequently had a suboccipital craniotomy and subtotal tumor excision due to morbid adherence to the brainstem. The histologic diagnosis was PA with Ki-67 <1% and negative for isocitrate dehydrogenase-1. There was a slow but progressive clinical improvement, and she has remained symptom-free for 4 years on follow-up.

Conclusion: PA in the elderly is a rare disease with distinct histologic and genetic peculiarities. This case review showed one of the oldest cases of cerebellar vermian PA presenting with recurrent spontaneous intratumoral hemorrhage, an extremely rare occurrence in benign glioma. Although complete surgical excision is recommended, partial resection is advocated for morbidly adherent tumors. Overall prognosis is worse in elderly PA.

Keywords: Elder; Intratumoral hemorrhage; Pilocytic astrocytoma; Vermian tumor.

Figure 1:

(a-c) Initial computed tomography (CT) scan and T2- and T2-star-weighted images showing a vermian mass lesion with associated intratumoral hemorrhage. (d) Enhanced T1-weighted image showing minimal enhancement of the tumor. (e) Fluid-attenuated inversion recovery image showing hydrocephalus. (f) CT scan 5 months later showing a repeat spontaneous intratumoral hemorrhage.

Figure 2:

(a-c) Postoperative computed tomography scan and fluid-attenuated inversion recovery image and T1-weighted image showing subtotal tumor excision.

Figure 3:

(a) Histology showing spindle and stellate-shaped cells with Rosenthal fibers and eosinophilic granular bodies scattered in the background. (b) Glial fibrillary acidic protein showing diffusely positive for the tumor cells.