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Table of Contents    
Year : 2011  |  Volume : 59  |  Issue : 2  |  Page : 276-280

Rosette-forming glioneuronal tumors: A report of two cases

1 Department of Neurosurgery, Apollo Health City, Hyderabad, India
2 Department of Pathology, Apollo Health City, Hyderabad, India

Date of Submission08-Jul-2010
Date of Decision12-Jul-2010
Date of Acceptance07-Aug-2010
Date of Web Publication7-Apr-2011

Correspondence Address:
Rahul Lath
Department of Neurosurgery, Apollo Health City, Hyderabad - 500 033
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.79148

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 » Abstract 

Rosette-forming glioneuronal tumor, a mixed glial and neuronal tumor, is a relatively new entity in tumors of the central nervous system, included in 2007 classification published by World Health Organization (WHO). It was initially described to occur in and around the fourth ventricle; however, recent case series have reported other locations also. Their occurrence in supratentorial and spinal locations has recently been reported. We report two cases of rosette-forming glioneuronal tumors, one in the midbrain and one in a suprasellar location, and review the literature.

Keywords: Glioneuronal tumor, rosette-forming, midbrain and suprasellar location

How to cite this article:
Sharma P, Swain M, Padua MD, Ranjan A, Lath R. Rosette-forming glioneuronal tumors: A report of two cases. Neurol India 2011;59:276-80

How to cite this URL:
Sharma P, Swain M, Padua MD, Ranjan A, Lath R. Rosette-forming glioneuronal tumors: A report of two cases. Neurol India [serial online] 2011 [cited 2019 Dec 9];59:276-80. Available from:

 » Introduction Top

Rosette-forming glioneuronal tumor (RGNT), initially described as dysembryoplastic neuroepithelial tumor (DNET) of the cerebellum, was considered as a separate entity based on distinctive morphology, location, age distribution and biologic behavior. [1] About 45 cases have been reported so far, and recently there has been a suggestion to label them as infratentorial or posterior fossa glioneuronal tumors. [2],[3],[4] This tumor predominantly affects young adults; typically arises in the midline; involves the cerebellum, wall or floor of the fourth ventricle and/or cerebral aqueduct; and may show parenchymal extension. [5],[6] We describe 2 cases of RGNT, one in the midbrain and one in a suprasellar location, and review the literature.

 » Case Reports Top

Case 1

A 16-year-old girl presented with complaints of diplopia and headache for the past 4 years. She was evaluated elsewhere and was started on antitubercular therapy (ATT) for a brainstem lesion suspected to be of tuberculous pathology based on radiological findings. During her follow up period, repeated scans showed no response to ATT, instead increase in size of the lesion was noted. At presentation to us, she had no neurological deficits. Gaze and eye movements were normal. Magnetic resonance imaging (MRI) scan of the brain showed a well-defined mass in the tectal region of the midbrain, which was hypointense on T1-weighted image, hyperintense on T2-weighted image, and showed no enhancement on contrast images [Figure 1]. Fluid attenuated inversion recovery (FLAIR) image did not show significant perilesional edema. A computed tomography (CT) guided stereotactic biopsy was done via a transfrontal route. Histopathological examination showed tumor was biphasic, composed of sheets of moderately cellular astroglial proliferation with oval and spindled nuclei. The stroma was loose, and myxoid and microcystic changes were evident. At places the cells had piloid processes. Cells with small, round, regular nuclei with speckled chromatin were seen arranged as perivascular pseudo-rosette and true rosettes with central fibrillary matrix [Figure 2]a. Foci of cells resembling oligodendroglial cells were seen [Figure 2]b. There was no evidence of necrosis or mitotic figures. Immunohistochemistry with glial fibrillary acidic protein (GFAP) and synaptophysin was positive [Figure 2]c, while the molecular immunology Borstel number one (MIB-1) index was low (<1%). The features were consistent with those of a rosette-forming glioneuronal tumor. Postoperatively she received 57 Gy of intensity-modulated radiation therapy in 27 cycles. At the last follow-up, 6 months later, she was asymptomatic.
Figure 1: Axial T2-weighted MRI showing the hyperintense tumor located in the midbrain

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Figure 2a: H and E, x10 showing astroglial proliferation with spindle and piloid cells along with microcystic areas, rosettes (small arrows) and pseudo-rosette (black arrow)
Figure 2b: H and E, x20 showing astroglial proliferation with oligodendroglia-like cells
Figure 2c: Immunohistochemical x40 positivity for GFAP and synaptophysin

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Case 2

A 17-year-old boy presented with history of sudden loss of consciousness of few hours' duration and past history of headache for 1 year. On admission he was obtunded with a Glasgow Coma Score (GCS)of E1V2M5 (8/15). There were no focal neurological deficits. Initial CT scan of the brain showed a hyperdense lesion in the suprasellar and interpeduncular cistern with extension into the third ventricle and dilation of both lateral ventricles [Figure 3]a. MRI was done, which showed a suprasellar mass involving the hypothalamus and extending into the third ventricle with hydrocephalus [Figure 3]b. He underwent bilateral ventriculo-peritoneal shunt as an emergency procedure in view of his sensorium and bradycardia. Post procedure, he was symptomatically better and his sensorium improved. During further hospital stay, features of hypothalamic dysfunction were noticed as hyponatremia with high output, and a fluctuating level of sensorium was observed. Hormonal analysis showed low level of cortisol, and he received steroid replacement therapy. Right fronto-temporal craniotomy was done, and grayish vascular suckable tumor in the third ventricle was biopsied. The tumor showed area of diffuse astroglial proliferation with oval nuclei and fibrillary cytoplasm. Also seen were cells with small, round and regular nuclei with stippled chromatin arranged in a neurocytic rosette around delicate neuropil matrix [Figure 4]a and b. There was no evidence of necrosis, mitotic figure or endothelial proliferation. Immunohistochemistry with GFAP and synaptophysin was positive [Figure 4]c. MIB-1 index was low (<1%). Biopsy report was consistent with a diagnosis of rosette-forming glioneuronal tumor. The patient is under regular radiological and clinical follow-up and is asymptomatic. The residual tumor is small and is being followed up with serial imaging.
Figure 3a: Non-enhanced CT scan showing a suprasellar mass with intratumoral hemorrhage
Figure 3b: Sagittal contrast-enhanced MRI showing an isointense lesion in the suprasellar region extending into the third ventricle with peripheral contrast enhancement

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Figure 4a: H and E, x40 showing cells with regular round nuclei arranged in neurocytic rosette with delicate neuropil matrix
Figure 4b: H and E, x40 showing astroglial proliferation with myxoid and microcystic changes
Figure 4c: Immunohistochemistry x40 showing positivity for GFAP and synaptophysin

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 » Discussion Top

Rosette-forming glioneuronal tumor (RGNT) is a recently classified central nervous system tumor corresponding to WHO grade I and shares the new international classification of diseases for oncology (ICD-O) code 9509/1 with the papillary glioneuronal tumor (PGNT). [5],[6] Two large series, one by Komori et al. in 2002 [1] (11 patients) and the other by Shah et al. in 2009 [3] (6 patients), have been reported. To date, a total of 45 cases have been reported in the English literature [Table 1]. [1],[2],[3],[4],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21] RGNT is a slow-growing tumor of the fourth ventricular region. Its population-based incidence is unknown, and this tumor predominantly affects young adults with a mean age of 29.2 years, with the youngest being 6 years old; and the oldest, 59 years of age. [3] There is a female preponderance (female-to-male ratio, slightly more than 2:1). [2],[3],[4] It has not been described in a familial form; however, a case of RGNT in a neurofibromatosis type-1 patient was reported but no genetic link was identified. [7] It has also been recently described in a patient with dysgenetic tricho-rhinopharyngeal type I syndrome. [8] The most common presenting manifestations are headache and ataxia, followed by visual disturbances and vertigo. [6] Other features of posterior fossa lesions are also present. As described initially, they are midline lesions centered in the fourth ventricle; however, tumors primarily situated in the surrounding tectal/ pineal and aqueductal regions are also well documented. Multifocal lesions with fourth ventricular, vermian, dorsal pontine, mesencephalic and thalamic components have also been reported. [5],[6] The occurrence of RGNT outside of the posterior fossa is rare and includes the suprasellar region and spinal cord [Table 1].
Table 1: Summary of RGNTs reported in the English literature

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On CT scans, the lesions are midline, relatively well circumscribed, solid, cystic or mixed and usually enhance focally with contrast. [1],[6] MRI features are T1-isointensity/ hypointensity and T2-hyperintensity with no contrast enhancement. [1],[5],[6] Intratumoral hemorrhage, like what was seen in our second case, is rare and has been previously described in two other reports. [9],[10]

Histopathology shows 2 types of tissue. The first is a bland perivascular astroglial proliferation with spindle and piloid cells resembling pilocytic astrocytoma. The second component is composed of cells with small and regular nuclei and speckled chromatin forming perivascular pseudo-rosettes and miniature neurocytic rosettes with a delicate neuropil matrix. Significant cytologic atypia and mitotic activity are absent. On immunostaining, synaptophysin labels the neuropil matrix and perivascular pseudo-rosettes. [1],[5],[6] The astrocytic component is labeled by GFAP and S-100. [1],[6] MIB-1 proliferation index is typically low (ranging from 0.35% to 3.07%). [1],[6] The nature of the tumor is indolent, as evidenced by absence of aggressive histologic features and low MIB-1 proliferative index. [1] Although RGNTs are benign tumors with the possibility of surgical cure and favorable prognosis, their location complicates surgical removal and imparts a significant risk of neurologic injury. [6] A survey of 45 cases with follow-up (range, 2-84 months; mean, 22 months) reveals no instance of tumor regrowth when treated by gross total resection alone. One case of recurrence after 10 years of follow-up [11] and one death have been reported. [1]

One patient in the series by Komori et al. and 1 patient of ours received postoperative radiation. [1] We considered radiation in our first case as there was documented increase in the size of the lesion. Radical resection is best avoided because of the anatomic location of these tumors. Significant postoperative morbidity, particularly affecting multiple cranial nerves, is common after major surgical resection. Long-term follow-up and more case reports are required to form uniform guidelines. Extra-fourth-ventricular locations, although rare, are being increasingly reported, like in our 2 cases.

 » References Top

1.Komori T, Scheithauer BW, Hirose T. A rosette-forming glioneuronal tumor of the fourth ventricle: Infratentorial form of dysembryoplastic neuroepithelial tumor? J Surg Pathol 2002;26:582-91.   Back to cited text no. 1
2.Pimentel J, Resende M, Vaz A, Reis AM, Campos A, Carvalho H, et al. Rosette-forming glioneuronal tumor: Pathology case report. Neurosurgery 2008;62:162-3.   Back to cited text no. 2
3.Shah MN, Leonard JR, Perry A. Rosette-forming glioneuronal tumors of the posterior fossa. Report of 6 cases. J Neurosurg Pediatr 2010;5:98-103.  Back to cited text no. 3
4.Tan CC, Gonzales M, Veitch A. Clinical implications of the infratentorial rosette-forming glioneuronal tumor: Case report. Neurosurgery 2008;63:175-6.  Back to cited text no. 4
5.Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, et al. The 2007 WHO Classification of Tumours of the Central Nervous System. Acta Neuropathol 2007;114:97-109.  Back to cited text no. 5
6.Rosenblum MK. The 2007 WHO Classification of Nervous System Tumors: Newly Recognized Members of the Mixed Glioneuronal Group. Brain Pathol 2007;17:308-13.  Back to cited text no. 6
7.Scheithauer BW, Silva AI, Ketterling RP, Pula JH, Lininger JF, Krinock MJ. Rosette- forming glioneuronal tumor: Report of a chiasmal- optic nerve example in neuro - fibromatosis type 1: Special pathology report. Neurosurgery 2009;64:771-2.  Back to cited text no. 7
8.Joseph V, Wells A, Kuo YH, Halcrow S, Brophy B, Scott G, et al. The 'rosette-forming glioneuronal tumor' of the fourth ventricle. Neuropathology 2009;29:309-14.   Back to cited text no. 8
9.Li YM, Li WQ, Pan Y, Lu YC, Long NY, Tao XF, et al. Rosette-forming glioneuronal tumour of the fourth ventricle with previous intratumoural haemorrhage: Case report and review of the literature. J Int Med Res 2009;37:958-66.   Back to cited text no. 9
10.Marhold F, Preusser M, Dietrich W, Prayer D, Czech T. Clinicoradiological features of rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle: Report of four cases and literature review. J Neurooncol 2008;90:301-8.   Back to cited text no. 10
11.Jacques TS, Eldridge C, Patel A, Saleem NM, Powell M, Kitchen ND, et al. Mixed glioneuronal tumour of the fourth ventricle with prominent rosette formation. Neuropathol Appl Neurobiol 2006;32:217-20.   Back to cited text no. 11
12.Kuchelmeister K, Demirel T, Schlorer E, Bergmann M, Gullotta F. Dysembryoplastic neuroepithelial tumour of the cerebellum. Acta Neuropathol (Berl) 1995;89:385-90.  Back to cited text no. 12
13.Albanese A, Mangiola A, Pompucci A, Sabatino G, Gessi M, Lauriola L, et al. Rosette-forming glioneuronal tumour of the fourth ventricle: Report of a case with clinical and surgical implications. J Neurooncol 2005;71:195-7.  Back to cited text no. 13
14.Adachi J, Nishikawa R, Hirose T, Matsutani M. Mixed neuronal-glial tumor of the fourth ventricle and successful treatment of postoperative mutism with bromocriptine: Case report. Surg Neurol 2005;63:375-9.  Back to cited text no. 14
15.Johnson M, Pace J, Burroughs JF. Fourth ventricle rosette-forming glioneuronal tumor: Case report. J Neurosurg 2006;105:129-31.  Back to cited text no. 15
16.Vajtai I, Arnold M, Kappeler A, Jeless O, Lukes A, Mariani L, et al. Rosette-forming glioneuronal tumor of the fourth ventricle: Report of two cases with a differential diagnostic overview. Pathol Res Pract 2007;203:613-9.   Back to cited text no. 16
17.Anan M, Inoue R, Ishii K, Abe T, Fujiki M, Kobayashi H, et al. A rosette-forming glioneuronal tumor of the spinal cord: The first case of a rosette-forming glioneuronal tumor originating from the spinal cord. Hum Pathol 2009;40:898-901.  Back to cited text no. 17
18.Wang Y, Xiong J, Chu SG, Liu Y, Cheng HX, Wang YF, et al. Rosette-forming glioneuronal tumor: Report of an unusual case with intraventricular dissemination. Acta Neuropathol 2009;118:813-9.  Back to cited text no. 18
19.Luan S, Zhuang D, Sun L, Huang FP. Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle: Case report and review of literature. Clin Neurol Neurosurg 2010;112:362-4.  Back to cited text no. 19
20.Arai A, Sasayama T, Tamki M, Sakagami Y, Enoki E, Ohbayashi C, et al. Rosette-forming glioneuronal tumor of the fourth ventricle-case report. Neurol Med Chir 2010;50:224-8.  Back to cited text no. 20
21.Kinno M, Ishizawa K, Shimada S, Masaoka H, Doi M, Seyama S, et al. Glioneuronal tumour of the fourth ventricle: A report of two cases. Cytopathology 2010;21:194-7.  Back to cited text no. 21


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