Volume 7, Issue 3 (Summer 2021)                   Iran J Neurosurg 2021, 7(3): 153-158 | Back to browse issues page


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Ghosh T, Dey S. Third Ventricle Anaplastic Oligodendroglioma: A Case Report. Iran J Neurosurg 2021; 7 (3) :153-158
URL: http://irjns.org/article-1-264-en.html
1- Department of Neurosurgery, Armed Forces Medical College, Pune, India , tamajyoti@gmail.com
2- Department of Neurosurgery, Armed Forces Medical College, Pune, India
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1. Background and Importance
Oligodendroglioma (ODG) constitutes 0.9% to 4% of all brain tumors [1]. It is relatively rare in the pediatric age group constituting 6.5% of cases with a Mean±SD age of 12±6 years [2]. ODG is most common in frontal and temporal lobes; however rare cases of intraventricular ODG have been reported [1, 3, 4]. Most commonly, intraventricular ODG arises from the anterior part of lateral ventricles [5]. ODG in the third ventricle is extremely rare and only a few cases of anaplastic ODG in the lateral and third ventricles have been reported in the literature [6, 7, 8]. ODG infiltrates locally to meninges [9] and rarely has leptomeningeal spread [9, 10]. Anaplastic ODG constitutes 24% of all pediatric ODGs [11].

2. Case Presentation
Here we present a case of 15-month-old male child with normal milestones who presented with vomiting, irritability, and gait imbalance for the last two months. The patient was conscious and oriented but irritable. His pupils were bilaterally 3 mm reacting. Fundoscopy was suggestive of grade II papilledema. His neurological examination was within normal limits. His anterior and posterior fontanelles were closed. We evaluated him with contrast (magnetic resonance imaging) brain MRI with whole spine screening. It showed a 50×24×39 mm well-defined lobulated heterogeneously enhancing intraventricular mass epicentered in the third ventricle floor extending to the lateral and fourth ventricle with spinal drop metastasis dorsal 12 levels. Preoperatively, his COVID-19 RT-PCR (Reverse Transcriptase Polymerase Chain Reaction) was positive. Given the raised Intracranial Pressure (ICP) features and the patient’s COVID-19 positive status, he underwent right sided medium pressure Ventriculoperitoneal (VP) shunt. The patient’s irritability and gait imbalance improved postoperatively. His Cerebrospinal Fluid (CSF) study was normal and CSF tumor markers (Alpha-Fetoprotein [AFP], beta-Human Chorionic Gonadotrophin [beta-HCG], and Lactate Dehydrogenase [LDH]) were negative. The patient was planned for definitive surgery later and underwent a left anterior transcortical approach with gross total tumor excision. Intraoperatively, tumor was greyish red, vascular, soft, and suckable, occupying the third ventricle extending into the lateral ventricle and postero-inferior into the aqueduct of Sylvius. The brain tumor interface was ill-defined. The intraoperative frozen section was suggestive of features similar to ependymoma. A ventriculostomy catheter was placed in the third ventricle to facilitate CSF drainage. After the operation, the patient developed right-sided hemiparesis. He was extubated on Postoperative Day (POD) 2. The External ventricular Drain (EVD) was removed on POD 4. There were no features of diabetes insipidus. Post-operative contrast MRI showed gross total excision of the tumor with minimal residual in sella and no infarct. The patient developed multiple spikes of fever along with tachycardia from POD 7. On POD 10, the patient developed irritability, dystonic movements of the right upper and lower limb. Then we started injection of benztropine 1 mg for the patient twice daily after ruling out meningitis, to which he responded well. Histopathological Examination (HPE) suggested anaplastic oigodendroglioma- Glial Fibrillary Acidic Protein (GFAP) positive, olig-2 positive, INI1 retained, Isocitrate Dehydrogenase 1 (IDH1) mutant, synaptophysin positive, chromogranin negative, Epithelial Membrane Antigen (EMA) focal membranous positive, Ki67 20%, S100 positive, Alpha Thalassemia/mental Retardation X (ATRX) retained, P53 non-mutant, and 1p13q co-deletion positive. Histone gene and BRAF mutation were negative. The patient was given postoperative chemotherapy after 4 weeks comprising lomustine 110 mg/m2 orally on day 1 followed by procarbazine 60 mg/m2 orally from day 8 to day 21 along with the injection of vincristine 1.4 mg/m2 intravenously on day 8 and day 29 (a total of 4 cycles). The patient tolerated chemotherapy well. On follow-up, the patient was conscious and oriented. However, there is a regression of developmental milestones. His six-month follow-up contrast MRI shows no residual tumor but a new heterogeneously contrast-enhancing lesion in the left thalamic and subthalamic region, which can explain his dystonic movements during the postoperative period (Figures 1, 2, 3, 4, 5, 6, and 7). 

3. Discussion
Anaplastic oligodendroglioma is more common in the older age group [12]. Its five-year median survival period is 20% [13]. The intraventricular location of ODG is an extremely rare entity. In his literature review of 33 studies involving seventy patients, Zada et al. mentioned only 3 patients of anaplastic intraventricular ODG [1]. Similarly, Sherif M. Elwatidy et al. in their analysis of 42 cases of the lateral and third ventricle tumors reported only a single case of ventricular anaplastic oligodendroglioma in adults [6]. Few isolated case reports of intraventricular oligodendrogliomas exist in the adult population [7, 8]. No case report was present in the pediatric age group. High-grade ODG is known for its recurrences. Packer and colleagues [13] mentioned a young adult with a high- grade ODG in the fourth ventricle who developed a frontal lesion 21 months after surgery.
Similarly Zada et al. also reported a 22-year-old female who underwent gross total resection of lateral ventricle anaplastic ODG extending to the third ventricle and 21 days later was readmitted with recurrence on the same site [1]. Unlike lobar ODG, where a patient presents with seizure, intraventricular ODG presents with raised ICP features [1, 3, 4, 5, 8]. Histology of intraventricular oligodendroglioma is consistent with parenchymal location. [3, 5]. Our case showed features of a high- grade lesion such as vascular proliferation and focal necrosis. On Immunohistochemistry (IHC), the neoplasm was Epithelial Membrane Antigen (EMA) focally positive, and chromogranin A negative. CSF was negative for tumor markers, thereby ruling out other common pediatric intraventricular neoplasms such as ependymoma, germinoma and lymphoma. IHC is important as anaplastic ODG shares similar H&E features with other tumors like central neurocytoma and ependymoma [14]. Moreover, most of the case reports of intraventricular ODG were from the pre-IHC era; thus the possibility of other lesions being diagnosed as ODG could not be ruled out. The transcortical intraventricular approach is the most common surgery seen among intraventricular anaplastic ODG by Zada et al., and all 3 cases had postoperative recurrences and died [1]. In his study of 200 cases, Shaw et al. found CSF dissemination in 14 out of 165 cases of oligodendroglioma, but no other details were provided [15]. Beck and Russel in 1942 mentioned a case of a 29-year-old male with a left posterior ventricular tumor with diffuse spread over the spinal cord [10]. Pitt et al. reported a case of the fourth ventricle oligodendroglioma with spread along the whole length of the spinal cord up to the midbrain [16]. Natale et al. mentioned a 67-year-old male with intraventricular ODG presenting with cauda equina [17].

4. Conclusion
Intraventricular anaplastic oligodendroglioma is an extremely rare tumor in pediatric age, and our case is probably the only documented pediatric case. Patients usually present with signs of raised intracranial pressure due to hydrocephalus. Radiological features are non-specific and sometimes may mimic ependymoma. Immunohistochemistry plays a pivotal role in accurate diagnosis and further characterization of the tumor. Chromosomal analysis helps in confirming the diagnosis and predicting treatment outcomes. Gross total resection is the treatment of choice followed by adjuvant chemotherapy. Patients with intraventricular ODG have a propensity for spinal drop metastasis. These tumors have a high recurrence rate and poor prognosis; thus frequent postoperative follow-up is necessary.

Ethical Considerations
Compliance with ethical guidelines

Written consent has been obtained from the subjects.

Funding
This research did not receive any grant from funding agencies in the public, commercial, or non-profit sectors.

Authors' contributions
Conception and design: Tamajyoti Ghosh, Subir Dey; Data collection: Tamajyoti Ghosh; Data analysis and interpretation: Tamajyoti Ghosh; Drafting the article: Tamajyoti Ghosh; Critically revising the article: Subir Dey; Reviewing submitted version of manuscript: Tamajyoti Ghosh; Approving the final version of the manuscript: Tamajyoti Ghosh, Subir Dey.

Conflict of interest
The authors declared no conflict of interest.

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Type of Study: Case report | Subject: Brain Tumors

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