|Year : 2019 | Volume
| Issue : 1 | Page : 24-26
Ganglioneuroblastoma of the paracavernous region
Kanika Bansal1, Sandeep Vaishya2, Rakesh K Gupta3, Sunita Ahlawat4, Anusheel Munshi1
1 Department of Radiation Oncology, Fortis Memorial Research Institute, Gurgaon, Haryana, India
2 Department of Neurosurgery, Fortis Memorial Research Institute, Gurgaon, Haryana, India
3 Department of Radiology, Fortis Memorial Research Institute, Gurgaon, Haryana, India
4 Department of Pathology, Fortis Memorial Research Institute, Gurgaon, Haryana, India
|Date of Submission||18-Jun-2018|
|Date of Acceptance||03-Sep-2018|
|Date of Web Publication||3-Jun-2019|
Dr. Anusheel Munshi
Manipal Hospitals, Dwarka, New Delhi
Source of Support: None, Conflict of Interest: None
Brain tumors commonly seen in the third decade of life are germinomas, medulloblastomas, and gliomas. A 21-year-old male presented with an extra-axial mass lesion in the right paracavernous and cerebellopontine region of the brain. Postoperative histopathology was ganglioneuroblastoma (GNB). We present the first report of GNB of this region of the brain along with a literature review of the past cases.
Keywords: Cerebellopontine angle tumor, ganglioneuroblastoma, paracavernous tumor, radiotherapy
|How to cite this article:|
Bansal K, Vaishya S, Gupta RK, Ahlawat S, Munshi A. Ganglioneuroblastoma of the paracavernous region. Int J Neurooncol 2019;2:24-6
| Introduction|| |
Ganglioneuroblastomas (GNBs) are rare neoplasms of neuroectodemal origin which may occur anywhere in anatomic distribution of sympathoadrenal neuroendocrine system. Tumours with distinct neuronal features are classified as either neuroblastoma or ganglioneuroblastoma depending on the level of neuronal differentiation. Neuroblastomas are more undifferentiated tumours, while a GNB is a tumour that has both mature ganglion cells and neuroblasts together with intermediate forms. Cerebral GNB is defined as a CNS embryonal tumour characterized by poorly differentiated neuroepithelial cells and groups of neurocytic and ganglion cells. The most common sites of origin of GNB are the adrenal medulla, extra-adrenal retroperitoneum, and posterior mediastinum. Although very rare, the GNB may also occur at the central nervous system, mainly involving the cerebral hemisphere., In the reviewed literature, we identified 12 intracranial GNB cases. To the best of our knowledge, this is the first report of a ganglioneuroblastoma in cerebellopontine (CP) angle/para-cavernous location in adults.
| Case Report|| |
A 21-year-old male presented with complaints of imbalance while walking, headache, and vomiting, flashes of light, and double vision for 2 weeks. His family history and past history were unremarkable. Contrast-enhanced magnetic resonance imaging (MRI) brain revealed a large heterogeneous extra-axial mass lesion measuring approximately 5.4 cm × 4.5 cm × 3.8 cm in the right cerebellopontine (CP) angle with extension into paracavernous region and perimesencephalic cistern causing mass effect on the brainstem [Figure 1]a and [Figure 1]b. The patient underwent craniotomy by supratentorial (right fronto-temporo-zygomatic craniotomy) and infratentorial approach (retromastoid suboccipital craniotomy) with gross total excision of tumor. Histopathology revealed bits of tumor composed of spindle cells having fascicles and bundles intermingled with nests of round cells showing ganglion cells and nerve bundles. Mature ganglion cells were entrapped [Figure 2]a, [Figure 2]b, [Figure 2]c. On immunohistochemistry analysis, spindle cell component showed diffuse expression of S100, neurofilament protein expressed in entrapped nerve bundles and ganglion cells. Synaptophysin was expressed in round cells, while CD56 was expressed both in round and in spindle cells. Neu-N was expressed in few nonneoplastic entrapped ganglion cells. Chromogranin was not expressed. Ki67 labeling index was 8%. The final impression was ganglioneuroblastoma (GNB). Postoperative MRI brain showed small area of residual disease. Whole-body positron emission tomography–computed tomography was done to rule out systemic/extracranial extent of disease and it showed postoperative changes in the right CP angle with absence of fluorodeoxyglucose -avid disease in the rest of the body. The patient's treatment plan was jointly decided in the tumor board. The patient was treated with adjuvant chemoradiotherapy 52.5 Gy in 25 fractions over 5 weeks with concomitant temozolomide (75 mg/m2/day). This was followed by systemic chemotherapy with a combination of irinotecan 20 mg/m2 (D1 to D5) and temozolomide 100 mg/m2 (D1 to D5) every 4 weekly for six cycles. The patient has completed the first cycle of adjuvant chemotherapy and is clinically doing well.
|Figure 1: (a and b) A T1-weighted gadolinium-enhanced magnetic resonance imaging of the brain showing tumor in the right cerebellopontine angle|
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|Figure 2: Light microscopic features of the tumor. (a and b) Show a spindle cell tumor with round cells (neuroblastoma), (c) shows collection of ganglion cells|
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| Discussion|| |
Tumors with distinct neuronal features are classified as either neuroblastoma or GNB depending on the level of neuronal differentiation. Neuroblastomas are more undifferentiated tumors, while a GNB is a tumor that has both mature ganglion cells and neuroblasts together with intermediate forms. Cerebral GNB is defined as a CNS embryonal tumor characterized by poorly differentiated neuroepithelial cells and groups of neurocytic and ganglion cells. This rare tumor shows varying degrees of neuronal differentiation, but dystrophic ganglion cells are a prominent element. Similarly, histological examination of the present case of GNB showed nerve bundles with entrapped mature ganglion cells. The diagnosis, however, has to be supported by immunohistochemistry.
The most common sites of origin of GNB are the adrenal medulla, extraadrenal retroperitoneum, and posterior mediastinum and less common sites are the neck and pelvis. Although very rare, the GNB may also occur at the central nervous system, mainly involving the cerebral hemisphere., Feigin and Cohen reported the first case of adult intracranial GNB in 1977. Since then, 12 intracranial adult GNBs have been reported [Table 1].,,,,,,,,, In view of the paucity of the cases reported, the postoperative management of these tumors has been varied. Surgery has been the most frequently used initial treatment modality in limited cases in the literature. Most of these patients have been subjected to adjuvant radiotherapy (dose varying from 10.9 to 60 Gy). In our literature review, of 12 cases, 3 patients received radiotherapy with chemotherapy with temozolomide. While some authors have used a combination of local radiotherapy along with concurrent temozolomide, others have given radiotherapy followed by sequential chemotherapy with temozolomide or other agents. Interestingly, Akin et al. used radiotherapy alone for recurrent GNB with posttreatment MRI, suggesting more than 50% regression in mass lesion, indicating reasonable radiosensitivity of these lesions to therapeutic radiation. Earlier reports used craniospinal irradiation as well, but recent reports have preferred using focal radiotherapy alone. Schipper et al. treated two cases of frontal GNB with postoperative chemoradiotherapy (60 Gy in 30 fractions) with concomitant temozolomide (75 mg/m2/day), subsequently followed by 6-monthly cycles of temozolomide (200 mg/m2/day for 5 days, every 4 weeks). These two patients were disease free for a follow-up period of 12–14 months.
|Table 1: Previously reported cases of adult intracranial ganglioneuroblastoma|
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A review was carried out on all 50 published adult cases with GNB, located at various sites including the adrenal gland, mediastinum, retroperitoneal area, the brain parenchyma, or the spinal cord. Median age at the onset was 39 years, and 52% of patients were female. For extracranial locations, treatment usually consisted of surgery followed by radiotherapy and adjuvant chemotherapy. The median survival of cerebral GNB s was 14 months and did not differ from the general outcome of GNBs of all sites (12 months). Only two patients in this series had intracranial GNB.
In our patient, the first one ever reported with the CP/paracavernous location in the brain, we used concurrent radiotherapy and temozolomide followed by irinotecan and temozolomide. While the follow-up period of our patient is not long, the patient tolerated the treatment well and was doing clinically fine at last contact.
To summarize, we have reported a case of adult cerebral GNB which was managed by multimodality treatment of neurosurgery, radiotherapy, and chemotherapy. We recommend this multimodality treatment for effective management of these rare tumors.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]