Brain Tumor Res Treat.  2016 Apr;4(1):35-39. 10.14791/btrt.2016.4.1.35.

Hypothalamic Extraventricular Neurocytoma (EVN) in a Pediatric Patient: A Case of EVN Treated with Subtotal Removal Followed by Adjuvant Radiotherapy

• Cho M
• Joo JD
• Kim BH
• Choe G
• Kim CY

• KMID: 2165233
• DOI: http://doi.org/10.14791/btrt.2016.4.1.35

Abstract

Extra ventricular neurocytoma (EVN) is a rare brain tumor with histologic features similar with a central neurocytoma, but located outside of the ventricular system. In this study, we present an unusual case of hypothalamic EVN in a 14-year-old patient. The patient underwent subtotal removal and had tumor relapse. The patient was then treated using intensity modulated radiation therapy, and the tumor remained stable for 24 months. This case report may be important in that this is the first pediatric case of EVN located in the hypothalamic region. EVN has similar radiologic features with pilocytic astrocytomas and therefore a hypothalamic EVN may be misdiagnosed as a hypothalamic glioma. Also, the pathologic-radiologic-clinical correlation of EVN located in the hypothalamic area may be different from that of EVNs originating from other usual sites.

Keyword

Hypothalamus tumor; Neurocytomas

MeSH Terms

Adolescent
Astrocytoma
Brain Neoplasms
Glioma
Humans
Hypothalamic Neoplasms
Neurocytoma*
Radiotherapy, Adjuvant*
Recurrence

Figure

• 

  Fig. 1 The results of the Humphrey visual field exam (A) revealed "bitemporal hemianopsia", preoperatively. The same exam (B) was performed when regrowth of the tumor was confirmed, and showed the same pattern with almost complete right eye blindness. After radiotherapy, there was no aggravation of the visual field according to the Goldmann perimetry exam (C). However, the right eye was unable to perceive light.

• 

  Fig. 2 T1-gadolinium-enhanced axial, sagittal, and coronal MR images showing the well-demarcated and homogeneously enhanced mass located in the sella-suprasella-hypothalamic area. A: Preoperative images. B: Immediate postoperative images. C: Postoperative images taken 1 year after the surgery showing regrowth of the tumor. D: The last follow up taken 24 months after intensity modulated radiation therapy.

• 

  Fig. 3 Histopathology of the lesion resected during the craniotomy. A and B: Hematoxylin and eosin staining indicates a tumor of moderate cellularity with vascular proliferation. Immunohistochemistry demonstrates neoplastic cells exhibiting strong, diffuse immunoreactivity for (C) chromogranin and (D) synaptophysin.

Reference

1. Nishio S, Takeshita I, Kaneko Y, Fukui M. Cerebral neurocytoma. A new subset of benign neuronal tumors of the cerebrum. Cancer. 1992; 70:529–537.

2. Sweiss FB, Lee M, Sherman JH. Extraventricular neurocytomas. Neurosurg Clin N Am. 2015; 26:99–104.

3. Xiong Z, Zhang J, Li Z, et al. A comparative study of intraventricular central neurocytomas and extraventricular neurocytomas. J Neurooncol. 2015; 121:521–529.

4. Han L, Niu H, Wang J, et al. Extraventricular neurocytoma in pediatric populations: a case report and review of the literature. Oncol Lett. 2013; 6:1397–1405.

5. Patil AS, Menon G, Easwer HV, Nair S. Extraventricular neurocytoma, a comprehensive review. Acta Neurochir (Wien). 2014; 156:349–354.

6. Schroeder JW, Vezina LG. Pediatric sellar and suprasellar lesions. Pediatr Radiol. 2011; 41:287–298. quiz 404-5.

7. Alshail E, Rutka JT, Becker LE, Hoffman HJ. Optic chiasmatic-hypothalamic glioma. Brain Pathol. 1997; 7:799–806.

8. Yang GF, Wu SY, Zhang LJ, Lu GM, Tian W, Shah K. Imaging findings of extraventricular neurocytoma: report of 3 cases and review of the literature. AJNR Am J Neuroradiol. 2009; 30:581–585.

9. Koeller KK, Rushing EJ. From the archives of the AFIP: pilocytic astrocytoma: radiologic-pathologic correlation. Radiographics. 2004; 24:1693–1708.

10. Kane AJ, Sughrue ME, Rutkowski MJ, et al. Atypia predicting prognosis for intracranial extraventricular neurocytomas. J Neurosurg. 2012; 116:349–354.

11. Kawano H, Kimura T, Iwata K, et al. Atypical extraventricular neurocytoma in a 3-year-old girl: case report with radiological-pathological correlation. Childs Nerv Syst. 2015; 31:1189–1193.