Cerebellomedullary ganglioglioma: A case report and review of literature

Purav Patel; J Reginald; P Mahadev; VS Mallikarjuna; S Annapurneswari; M Balamurugan

doi:10.4103/1817-1745.32003

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CASE REPORT

Year : 2007 \| Volume : 2 \| Issue : 1 \| Page : 23-25

Cerebellomedullary ganglioglioma: A case report and review of literature

Purav Patel¹, J Reginald¹, P Mahadev², VS Mallikarjuna³, S Annapurneswari³, M Balamurugan¹
¹ Department of Neurosurgery, Apollo Speciality Hospital, Chennai, India
² Department of Radiation Oncology, Apollo Speciality Hospital, Chennai, India
³ Department of Pathology, Apollo Speciality Hospital, Chennai, India

Correspondence Address:
Purav Patel
Neurosurgery Department, Apollo Speciality Hospital, 320- Anna Salai, Chennai - 600 035
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/1817-1745.32003

Abstract

Posterior fossa gangliogliomas are uncommon primary neoplasms of the central nervous system. Only 34 cases have been reported in the literature; and out of these, only 3 cases have been studied with computerized tomography (CT) and magnetic resonance imaging (MRI). A case of a 13-year-old boy with cerebellomedullary ganglioglioma is presented with CT, MRI and histopathological findings.

Keywords: Central nervous system neoplasm, cerebellomedullary, ganglioglioma

How to cite this article:
Patel P, Reginald J, Mahadev P, Mallikarjuna V S, Annapurneswari S, Balamurugan M. Cerebellomedullary ganglioglioma: A case report and review of literature. J Pediatr Neurosci 2007;2:23-5

How to cite this URL:
Patel P, Reginald J, Mahadev P, Mallikarjuna V S, Annapurneswari S, Balamurugan M. Cerebellomedullary ganglioglioma: A case report and review of literature. J Pediatr Neurosci [serial online] 2007 [cited 2023 Dec 3];2:23-5. Available from: https://www.pediatricneurosciences.com/text.asp?2007/2/1/23/32003

Introduction

Gangliogliomas are unusual central nervous system (CNS) tumors in children and young adults.^{[1],[2],[3],[4]} They represent 0.4-1.7% of all CNS tumors.^{[2],[3],[4],[5]} They are composed of differential nerve cells, in the form of ganglion cells, with a glial background that is typically astrocytic.^[1],[2],[3] Cerebral hemispheres are more commonly involved; followed by third ventricle, cerebellum, brain stem and spinal cord.^[1],[3],[4] Histologically, the diagnosis requires both astrocytic and neuronal cell populations to be present.^{[1],[5],[6],[7]} Only 34 cases are documented in the published literature; and out of these, only 3 cases have been studied with CT and MRI.^{[1],[2],[3],[4]} We present this rare entity with imaging and comprehensive histopathological features.

Case Report

A 13-year-old Arabian boy presented with a history of change in voice since 2 years, nasal regurgitation since 1 month with breathing difficulty on and off and dysphagia since 1 week. Clinical examination revealed high-pitched nasal quality of voice (as a consequence of soft palate dysfunction) and horizontal jerky nystagmus more on looking towards the left side with the fast component on the same side. Examination revealed diminished left corneal reflex. Gag reflex was absent bilaterally, with tongue and uvula deviated towards the left side. Computerized tomography (CT) scan brain showed an ill-defined isodense to hypodense mass in the dorsal cerebellomedullary region extending into foramen magnum with central contrast enhancement [Figure - 1]A, B. To better visualize anatomic relationship, an magnetic resonance imaging (MRI) brain was done, which showed a solid-cystic mass in the cerebellomedullary region extending into the foramen magnum, displacing the roof of the fourth ventricle to the right side. T1-weighted images showed a spectrum of signal ranging from isointense to hypointense, relative to surrounding neural tissue [Figure - 2]A, [Figure - 3]A, and areas of contrast enhancement with gadolinium [Figure - 2]B, [Figure - 3]B, [Figure - 4]. T2-weighted images showed high signal intensity. The heterogenous cystic and solid appearance on MRI with extension into foramen magnum prompted for decompression and radical excision. Suboccipital craniectomy was done. Intraoperatively the tumor was firm and adherent to surrounding normal cerebellomedullary tissue. Attempted excision led to transient cardiac dysrhythmias; therefore, total excision was not attempted. Histopathology revealed spindle-shaped glial cells with spindle- to oval-shaped nuclei in fascicles. Ganglion cells had large vesicular nuclei with prominent nucleoli and occasional bizarre and binucleate forms [Figure - 5]. Immunohistochemical study revealed positive stain for S100 [Figure - 6] proteins, synaptophysin [Figure - 7] and neurofilament [Figure - 8]. Glial component showed positivity for GFAP [Figure - 9]. On the basis of classical histopathological findings with supplementary immunohistochemical evidence, diagnosis of ganglioglioma was arrived at. After obtaining informed consent, 3D conformal radiation therapy with a dose of 5,400 CGy was given to the tumor bed over 6 weeks. Lower cranial nerves function improved. There was no difficulty in breathing or swallowing, and it was possible to elicit gag reflex at the end of radiation treatment. As the patient was not from India, we lost follow-up of the patient.

Discussion

Courville in 1930 coined the term "ganglioglioma" for tumors arising from undifferentiated cells that became neoplastic yet eventually differentiated along both glial and neuronal lines to achieve the mature form of the cells.^[1],[7] Temporal lobe is the most common site of involvement.^[4] Patients usually have a long clinical history, with predominance of seizures.^[2],[4] Posterior fossa gangliogliomas usually have a benign clinical course compared to other intrinsic brain stem tumors.^[2],[3],[4] CT appearance is variable. A region of low density or isodensity compared with normal brain stem with areas of contrast enhancement and calcification is present.^[2],[3],[4] MRI shows decreased signal relative to surrounding neural tissue on T1-weighted images that are readily distinguished from CSF.^[2],[3],[4] On T2-weighted image, discrete areas of increased intensity and swollen gyri on the cortical margin are usual findings.^[2],[3],[4] Histologic diagnosis may be complicated due to difficulty in determining whether the ganglion cells are truly neoplastic or simply "trapped" neurons in an infiltrating glioma.^[3],[5],[7] According to Russel and Rubinstein, neoplastic neurons must be either clearly heterotopic (located away from gray matter) or atypical, showing disorientation, bizarre shapes or sizes or binucleation.^[1],[3],[6] Stainings with synaptophysin and neurofilament protein markers are not pathognomonic for glioneuronal tumors and must be interpreted with caution whenever other histologic or ultra-structural evidence of neuronal differentiation is lacking.^[5],[6] Usefulness of radiotherapy in ganglioglioma is not clear, and long-term survival after surgical excision without adjuvant radiotherapy is well documented in the published literature.^[2],[4] But in this case as the tumor was situated in the posterior fossa and only partial excision was possible, it was decided to give external beam radiotherapy.

Cerebellomedullary gangliogliomas are rare. In children ganglioglioma should be considered as a differential diagnosis when MRI reveals a heterogenous contrast-enhancing mass with or without cystic lesion in the cerebellomedullary region. Immunohistochemical markers form an adjuvant for histopathological diagnosis.

Acknowledgments

We thank Prof. K. Ganapathy, Senior Consultant Neurosurgeon, Apollo Hospitals, Chennai, for reviewing the article and providing useful suggestions.

References

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7.	Russell DS, Rubinstein LJ. Pathology of Tumors of the Nervous System. 5th ed. revised by Rubinstein LJ. Williams and Wilkins: Baltimore; 1989. p. 289-306.