Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 58, Num. 3, 2010, pp. 477-480

Neurology India, Vol. 58, No. 3, May-June, 2010, pp. 477-480

Case Report

Intraventricular ganglioglioma with bleed: A rare case report

Dhananjaya I Bhat¹, Anita Mahadevan², Ranjan Manish¹, Somanna Sampath¹, BA Chandramouli¹, SK Shankar²

¹ Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore - 560 029, India
² Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore - 560 029, India
Correspondence Address: Dhananjaya I Bhat, Associate Professor, Department of Neurosurgery, National Institute of Mental Health & Neurosciences, Bangalore 560 029, India, dibhat@rediffmail.com

Date of Acceptance: 08-Jun-2010

Code Number: ni10121

PMID: 20644285
DOI: 10.4103/0028-3886.65924

Abstract

Gangliogliomas are benign lesions that are predominantly temporal in location and present with chronic epilepsy. Intraventricular gangliogliomas are extremely rare and still rarer is intratumoral hemorrhage. Till date only 9 cases of lateral ventricular gangliogliomas have been reported. To the best of our knowledge only 1 case of intratumoral hemorrhage has been reported. We report a rare case of lateral ventricular ganglioglioma with intratumoral hemorrhage in a 27-year-old man.

Keywords: Angioma, ganglioglioma, hemorrhage, intraventricular, synaptophysin

Introduction

Gangliogliomas are benign lesions that are predominantly temporal in location and present with often intractable seizures. Intraventricular location mimicking central neurocytoma is very rare. Only 9 cases have been reported in the literature till 2009. ^[1] We report here, a rare case of lateral ventricular ganglioglioma with intratumoral hemorrhage.

Case Report

A 27-year-old man presented with a history of rapidly progressing severe headache with vomiting of 20 days duration, associated with blurring of vision and decreased recent memory of 15 days. Neurologic examination revealed papilledema and bilateral sixth nerve palsy. Cranial imaging, both computed tomography (CT) and magnetic resonance imaging (MRI), showed an intraventricular lesion arising from the septum occupying the body of both the lateral ventricles causing obstructive hydrocephalus. The mass was predominantly hyperdense on plain CT scan [Figure - 1]. On MRI image, the lesion was heterointense with multiple small cysts, iso- to hypointense on T1-weighted images with areas of hypo- and hyperintensities on T2-weighted images suggestive of a lesion with subacute bleed [Figure - 2]a and b. He underwent an interhemispheric transcallosal approach and total excision of the lesion [Figure - 3]. The lesion was hemorrhagic, firm, grayish, and attached to the septum. Histopathology examination of the resected mass showed a tumor with large focus of central hemorrhage walled in by tumor tissue of low cellularity with biphasic loose and compact glial zones [Figure - 4]a. The central hemorrhagic zone was bordered by a leash of telangiectatic vessels along the periphery resembling an angioma [Figure - 4]b. Calcification and ferrugination of small vessels were seen along the fibrous capsule of the hematoma [Figure - 4]c, reflecting the chronic nature of the lesion. The tumor cells had round to elongate bare vesicular nuclei dispersed in a fibrillary background with several eosinophilic granular bodies [Figure - 4]d. The cells showed biphenotypic differentiation with several cells expressing glial fibrillary acid protein within the cytoplasm and fibrillary processes [Figure - 4]e, whereas others revealed a nuclear and cytoplasmic expression for the neuronal marker, Neu N [Figure - 4]f. In addition, scattered large uni- or binucleate ganglionic cells with eccentric nuclei and prominent nucleoli [Figure - 4]g that revealed aberrant membrane localization of synaptophysin were seen [Figure - 4]h. These ganglionic cells showed dystrophic intracytoplasmic accumulation of the phosporylated neurofilament [Figure - 4]i. The mitotic activity was very low (MIB-1 labeling index of 1%). The features were characteristic of a ganglioglioma with angiomatous component causing intratumoral hemorrhage.

Postoperatively, the patient had a wound bulge and became progressively drowsy. Repeat CT scan showed ventriculomegaly for which a right ventriculoperitoneal shunt was done. Following surgery, he had improvement in sensorium and at discharge from the hospital, he was conscious, and alert with improvement in recent memory. He was not subjected to any adjuvant therapy, as the tumor had been totally excised.

Discussion

Gangliogliomas are rare central nervous system tumors and account for approximately 2% of all the brain tumors. They are composed of dysplastic neuronal and neoplastic glial components. Most of the patients present in the first 3 decades of life with chronic epilepsy. Although the most frequent intracranial location is the temporal lobe, other locations in order of frequency include frontal, parietal, and occipital lobes, and rarely in the brain stem, optic chiasm, pineal region, and spinal cord. ^[2] A lateral ventricular location, however, is extremely rare and the first case report was by Majos and colleagues in 1998. ^[3] Till 2009, only 9 cases of lateral ventricular ganglioglioma have been described. ^[1] Neuroimaging of ganglioglioma shows a solid (43-50%), cystic (5%), or solid-cystic (32-52%) mass lesion typically located in the periphery of cerebral hemispheres. On an average about half of these tumors have cysts, calcifications, and enhancement, but hemorrhage is rare and has been reported only once. ^[2],[4] Interestingly, our patient had large areas of bleed, identified on imaging as well as on histopathology.

Grossly gangliogliomas are solid grayish, firm, and granular or cystic with an admixture of glial cells and neurons. The ganglion cells are recognized by their large size, bizarre shape, vesicular nuclei, and prominent nucleoli with positive staining for synaptophysin or neurofilament protein. The neoplastic glial cells may be either astrocytic or oligodendrocytic that dictate the grading and biologic behavior of the tumor. These tumors are most often Grade I, malignant transformation with mitotic activity, and necrosis being rare. ^[2] In the present case, numerous telangiectatic vessels resembling an angioma were seen bordering the hematoma, which might be responsible for the intratumoral hemorrhage. In intraventricular locations, subependymal giant cell astrocytomas are commonly associated with hemorrhage from the angiomatous component that often accompanies the tumor. Although an angiomatous component was identifiable, characteristic giant cells with variable glial and neuronal expression as seen in subependymal giant cell astrocytoma were not seen in our case. Furthermore, eosinophilic granular bodies accompanying the pilocytic astrocytoma component of the tumor is not a feature of subependymal giant cell astrocytomas.

The role of postoperative adjuvant radiotherapy for these benign tumors is controversial and usually reserved for those with disease progression, malignant lesions, or for recurrences that are not amenable for surgery. ^[4]

References

Copyright 2010 - Neurology India

The following images related to this document are available:

Photo images