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Year : 2020  |  Volume : 3  |  Issue : 2  |  Page : 101-103

Primary cerebral neuroblastoma in young adult

Department of Radiation Oncology, Basavatarakam Indo-American Cancer Hospital and Research Institute, Hyderabad, Telangana, India

Date of Submission07-Aug-2020
Date of Acceptance29-Nov-2020
Date of Web Publication26-Dec-2020

Correspondence Address:
Dr. Deleep Kumar Gudipudi
Department of Radiation Oncology, Basavatarakam Indo-American Cancer Hospital and Research Institute, Road Number 10, Banjara Hills, Hyderabad - 500 034, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJNO.IJNO_19_20

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Neuroblastoma is an embryonal malignancy of the sympathetic nervous system arising from pluripotent sympathetic cells (neuroblasts). It is the most common extracranial solid tumor in infants. Although it can arise anywhere along sympathetic chain, it is extremely unusual for neuroblastoma to present as intracranial lesion and that too in a young adult. Here, we describe a 24-year-old male presented with complaints of headache and retro-orbital pain. Magnetic resonance imaging (MRI) of the brain revealed a left occipital lobe mass lesion. He underwent left parieto-occipital craniotomy and near-total excision. Postoperative MRI showed minimal residual lesion. Initial histopathological examination was reported as a malignant round cell tumor. However, block and slide review with immunohistochemistry markers was suggestive of neuroblastoma. He received adjuvant radiation of 50.4 Gy in 28 fractions, followed by eight cycles of adjuvant chemotherapy with lomustine and cisplatin. At 3-year follow-up, MRI showed post-operative gliosis in left parietal lobe with no evidence of recurrence.

Keywords: Intracranial neoplasm, Neu-N positive, primary cerebral neuroblastoma, rare intracranial malignancy, sympathetic nervous system neoplasm

How to cite this article:
Selvaraj VK, Gudipudi DK. Primary cerebral neuroblastoma in young adult. Int J Neurooncol 2020;3:101-3

How to cite this URL:
Selvaraj VK, Gudipudi DK. Primary cerebral neuroblastoma in young adult. Int J Neurooncol [serial online] 2020 [cited 2023 Jun 1];3:101-3. Available from: https://www.Internationaljneurooncology.com/text.asp?2020/3/2/101/305061

  Introduction Top

Neuroblastoma accounts for about 6% of all pediatric malignancies. It is the most common extracranial solid tumor in infants and occurs predominantly in the retroperitoneum.[1] Metastases to bone, lymph nodes, and liver are seen in more than half of the patients.[2] It is uncommon for neuroblastoma to metastasize to brain.[3] To present as a primary lesion in the cerebral hemisphere is extremely rare, that too it has never been reported in a young adult. Here, we describe such an extremely rare presentation of primary cerebral neuroblastoma in a 24-year-old male in the left occipital lobe and treatment modalities adopted.

  Case History Top

A 24-year-old male presented with complaints of headache and left retro-orbital pain for 2 weeks. On examination, there was no focal neurological deficit. Magnetic resonance imaging (MRI) brain revealed a 5.5 cm × 4.1 cm mass lesion in left occipital region with calcifications. Hence, he underwent left parieto-occipital craniotomy and near-total excision of left occipital lesion elsewhere. Postoperative MRI showed an irregular lesion of 3 cm × 2.8 cm with minimal heterogeneous contrast enhancement in the left occipital lobe, suggestive of minimal residual lesion with postoperative hemorrhagic to gliotic areas [Figure 1]a, [Figure 1]b. Cerebrospinal fluid (CSF) cytology had no viable cells. Whole-body positron emission tomography-computed tomography scan showed a heterogeneously enhancing metabolically active lesion in the left parieto-occipital region, suggestive of residual disease with postoperative changes.
Figure 1: Postoperative magnetic resonance imaging brain showing irregular hypointense residual lesion (3 cm × 2.8 cm) with hyperintense areas and minimal heterogeneous contrast enhancement in left occipital lobe. (a) T1-weighted images, axial section. (b) T1-weighted images, sagittal section

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Initial histopathological examination of the surgical specimen was reported to be malignant round cell tumor. Blocks and slide reviews with immunohistochemistry (IHC) markers revealed Neu-N diffusely positive, glial fibrillary acidic protein focally positive, and p53 negative. Hence, a diagnosis of primary cerebral neuroblastoma was considered.

In view of the rarity of the disease, extensive literature review and multidisciplinary tumor board discussion was done. In view of localized disease confined to the central nervous system (CNS), the patient was planned for adjuvant radiotherapy followed by chemotherapy. He received external beam radiotherapy to the left occipital lobe; a total dose of 50.4 Gy in 28 fractions was delivered at 1.8 Gy per fraction using 6 MV photons by Rapid-Arc intensity-modulated radiotherapy technique. He tolerated radiation treatment well, following which he received eight cycles of adjuvant chemotherapy of lomustine 120 mg orally and cisplatin 150 mg intravenously. At 3-year follow-up, the patient is asymptomatic, and MRI showed no evidence of relapse [Figure 2]a, [Figure 2]b.
Figure 2: Follow-up magnetic resonance imaging brain showing irregular hypointense postoperative gliotic areas with no significant contrast enhancement in the left occipital lobe. (a) T1-weighted images, axial section. (b) T1-weighted images, sagittal section

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  Discussion Top

Primary cerebral neuroblastoma is often considered to be a primitive neuroectodermal tumor (PNET) showing neuronal differentiation. Neuroblastoma comprises 6% of all PNETs in CNS and generally occurs during the first decade of life as supratentorial lesions.[4] Frontal and parietal lobes are more common sites to be involved.[5],[6],[7] According to Horten and Rubinstein, primary CNS neuroblastoma occurs approximately in one case per decade.[5] The case reported here is unique in two ways that it has occurred in the occipital lobe and in young adult.

The symptoms of primary cerebral neuroblastoma are associated with raised intracranial pressure, similar to most CNS tumors. The tumors are usually large, well circumscribed associated with areas of cysts, necrosis, or focal hemorrhage.[5],[6],[7] The characteristic histologic features of neuroblastoma are Homer-Wright rosettes, ganglionic differentiation, and argyrophilic cells process in silver impregnation.[4],[5],[8] Based on degree and extent of fibrous connective tissue, three variants have been described: classical, transitional, and desmoplastic types. The connective tissue stroma is limited, intermediate, and prominent in classical, transitional, and desmoplastic subtypes, respectively.[4],[5],[8] In addition to the histological findings, IHC markers are used to confirm the cell of origin of tumors. In our case, Neu-N marker was positive, depicting neuronal differentiation in the lesion.

Treatment of primary cerebral neuroblastoma is primarily surgical resection of the tumor. The extent of resection is an important prognostic factor. However, in the majority of cases, total resection is not possible due to the large size of the tumor.[5],[6],[7],[9] Adjuvant radiotherapy was considered in the majority of cases and shown to improve local control.[5],[6],[7],[10],[11] It is a common hypothesis that primary CNS neuroblastomas behave similar to medulloblastomas and tend to have higher neuroaxis dissemination.[5],[6],[7],[10] Thus, some authors recommended routine inclusion of craniospinal axis during adjuvant radiation,[5],[10] while others included craniospinal axis only when spinal seeding was present in CSF cytology or MRI.[7] In our patient, CSF cytology was negative; hence, we avoided craniospinal irradiation. Chemotherapy regimens consisting of nitrosoureas, cisplatin, etoposide, MOPP (nitrogen mustard, vincristine, procarbazine, and prednisolone) etc., were tried and proved to be beneficial, especially in patients for whom subtotal tumor resection was done.[7] Combined treatment modality has proved to result in improved survival rates.[9],[11] Based on the above recommendations in literature, our patient was effectively treated with adjuvant radiotherapy followed by chemotherapy.

  Conclusion Top

Primary cerebral neuroblastoma is an extremely rare malignant neoplasm of CNS. A thorough histopathological examination with IHC markers is warranted to rule out primary neuroblastoma in all intracranial supratentorial neoplasms of infancy, childhood, and even in young adults. Based on literature review and our case report, maximal safe resection with adjuvant radiotherapy followed by chemotherapy provides durable disease control.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Grovas A, Fremgen A, Rauck A, Ruymann FB, Hutchinson CL, Winchester DP, et al. The National Cancer Data Base report on patterns of childhood cancers in the United States. Cancer 1997;80:2321-32.  Back to cited text no. 1
DuBois SG, Kalika Y, Lukens JN, Brodeur GM, Seeger RC, Atkinson JB, et al. Metastatic sites in stage IV and IVS neuroblastoma correlate with age, tumor biology, and survival. J Pediatr Hematol Oncol 1999;21:181-9.  Back to cited text no. 2
Matthay KK, Brisse H, Couanet D, Couturier J, Bénard J, Mosseri V, et al. Central nervous system metastases in neuroblastoma: Radiologic, clinical, and biologic features in 23 patients. Cancer 2003;98:155-65.  Back to cited text no. 3
Becker LE, Hinton D. Primitive neuroectodermal tumors of the central nervous system. Hum Pathol 1983;14:538-50.  Back to cited text no. 4
Horten BC, Rubinstein LJ. Primary cerebral neuroblastoma. A clinicopathological study of 35 cases. Brain 1976;99:735-56.  Back to cited text no. 5
Bennett J, Rubinstein LJ. The biological behaviour of primary cerebral neuroblastoma: A reappraisal of the clinical course in a series of 70 cases. Ann Neurol. 1984;16:21-27.  Back to cited text no. 6
Berger MS, Edwards MS, Wara WM, Levin VA, Wilson CB. Primary cerebral neuroblastoma. Long-term follow-up review and therapeutic guidelines. J Neurosurg 1983;59:418-23.  Back to cited text no. 7
Dehner LP, Abenoza P, Sibley RK. Primary cerebral neuroectodermal tumors: Neuroblastoma, differentiated neuroblastoma, and composite neuroectodermal tumor. Ultrastruct Pathol 1988;12:479-94.  Back to cited text no. 8
Albright AL, Wisoff JH, Zeltzer P, Boyett J, Rorke LB, Stanley P, et al. Prognostic factors in children with supratentorial (nonpineal) primitive neuroectodermal tumors. A neurosurgical perspective from the Children's Cancer Group. Pediatr Neurosurg 1995;22:1-7.  Back to cited text no. 9
Gaffney CC, Sloane JP, Bradley NJ, Bloom HJ. Primitive neuroectodermal tumours of the cerebrum. Pathology and treatment. J Neurooncol 1985;3:23-33.  Back to cited text no. 10
Cohen BH, Zelter PM, Boyett JM, Geyer JR, Allen JC, Finlay JL, et al. Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: A Children's Cancer Group randomized trial. J Clin Oncol 1995;13:1687-96.  Back to cited text no. 11


  [Figure 1], [Figure 2]


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