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LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 3  |  Page : 323-325

Dysembryoplastic neuroepithelial tumor transforming into malignancy: A case report


1 Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission31-Jan-2014
Date of Decision03-Mar-2014
Date of Acceptance30-May-2014
Date of Web Publication18-Jul-2014

Correspondence Address:
Ashish Aggarwal
Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.137011

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How to cite this article:
Aggarwal A, Salunke P, Sodhi HB, Vasishta R K, Gowda KK. Dysembryoplastic neuroepithelial tumor transforming into malignancy: A case report. Neurol India 2014;62:323-5

How to cite this URL:
Aggarwal A, Salunke P, Sodhi HB, Vasishta R K, Gowda KK. Dysembryoplastic neuroepithelial tumor transforming into malignancy: A case report. Neurol India [serial online] 2014 [cited 2019 Dec 15];62:323-5. Available from: http://www.neurologyindia.com/text.asp?2014/62/3/323/137011


Sir,

The term dysembryoplastic neuroepithelial tumor (DNET) was first coined by Daumas-Duport and colleagues to describe a cortical lesion presenting in childhood. [1] The World Health Organization (WHO) has categorized it under grade 1 tumors. However, there have been isolated case reports of malignant transformation of a previously histologically proven DNET. The present case is the fifth such report, but the first report of gemistiocytic transformation.

A 29-year-old male presented with history of complex partial seizures since childhood. Neurological examination was essentially normal. Computed tomography (CT) scan had revealed a hypodense non-contrast enhancing lesion in the left frontal lobe [Figure 1]e. Magnetic resonance imaging (MRI) showed a non-contrast enhancing space occupying lesion in left frontal lobe [Figure 1]a-d. Intraoperatively, the tumor was found on the surface-pale-looking, firm, minimally vascular with a poor plane of cleavage between tumor and normal brain. Postoperative CT revealed gross total excision of tumor [Figure 1]f. Histology of the tumor confirmed the diagnosis of DNET-complex type [Figure 3]a and b. Patient did not receive postoperative radiotherapy or chemotherapy. He was seizure-free on antiepileptic drugs. Approximately one year after first surgery, he presented with features suggestive of raised intracranial pressure. Examination showed bilateral pappiledema and right-sided spastic hemiparesis (grade 4/5) and right lower motor neuron type facial paresis. MRI showed a hypointense lesion in left cingulate gyrus and corpus callosum and going across the midline [Figure 2]a-e. In contrast to earlier lesion (DNET), this lesion was intensely contrast-enhancing. Patient underwent second surgery. The tumor was grayish, soft, suckable, and moderately vascular. Subtotal excision could be achieved [Figure 2]f Postoperative period was uneventful. Histopathological Examination was astrocytoma grade 2 with gemistocytic component [Figure 3]c and d. In view of subtotal excision and gemistocytic component, patient received radiotherapy. At 6 months follow-up, patient was conscious with same deficits as in the preoperative period.
Figure 1: Radiology Images before first surgery. (a) Magnetic resonance imaging (MRI) T1WI plain. Axial section showing a hypointense lesion in the left frontal lobe. (b-d) MRI T1WI Contrast. Axial section (b), Sagittal section (c), and Coronal section (d) showing non-contrast-enhancing lesion in the left frontal lobe. (e) Contrast CT scan showing non-contrast-enhancing lesion in the left frontal lobe and (f) postoperative plain CT showing gross total excision of tumor

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Figure 2: Radiology Images before second surgery. (a) MRI T1WI plain. Axial section showing a hypointense lesion in the region of the left cingulate gyrus and corpus callosum. (b-d) MRI T1WI Contrast. Axial section (b), Coronal section (c), and Sagittal section (d) showing intense contrast enhancement in the lesion in the region of the left cingulate gyrus and corpus callosum. (e) MRI coronal fl air image showing hyperintense lesion. (f) Postoperative plain CT showing subtotal excision of tumor

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Figure 3: Histopathology images.( a and b) after fi rst surgery, (c and d) after second surgery. (a) (hematoxylin and eosin; h and e, ×400) Shows mucin-filled microcyte (indicated by a star), oligodendrocyte-like cells (arrow head) and floating neuron (arrow head) (b) (×400) neurons (arrow head) showing glial fibrillary acidic protein (GFAP) positivity.
(c) (H and E, ×200). Areas of tumor with predominance of gemistocytes. (d) (GFAP, ×200). Gemistocytes showing positivity for GFAP


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It has been a quarter of a century since these intriguing lesions known as DNET were first described for relatively benign lesions, which were thought to be cortical dysplasias situated on the margin of benign neoplasia. [1],[2] According to Daumas-Duport and colleagues, the criteria for the diagnosis of DNET should include: (1) partial seizures, with or without secondary generalization beginning before age 20 (2) no neurological deficit or presence of a stable and likely congenital neurological deficit (3) cortical topography of the lesion as best demonstrated on MRI, and (4) no mass effect on CT or MRI (except if related to a cyst). [2] There have been isolated case reports of astrocytic (malignant) transformation of a histologically proven DNET. [3],[4],[5] The present case is probably the fifth reported case in the literature of such a transformation, but perhaps the first one with gemistocytic differentiation. The mechanism of development of a gemistocytic astocytoma in a proven DNET remains a matter of debate. Its proposed mechanism is as either a collision tumor or the neoplastic change within DNET. [4] Classically, DNETs have been described to have a benign course with cortical dysplasia rather than true neoplasias. However, these reports of malignant transformation lend credence to the theory that there is at least a subgroup of lesions amongst DNETs, which have a malignant behavior and require adjunct treatment strategies. The issue is to identify this specific subset. How to do this? Whether some special characteristics on histopathologic examination or the genetic analysis hold the key remains unanswered. Till then, the need for lifelong surveillance in a case of DNET cannot be over emphasized.

 
  References Top

1.Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, Laws ER Jr, Vedrenne C. Dysembryoplasic neuroepithelial tumor: A surgically curable tumor of young patients with intractablepartial seizure. Report of thirty-nine cases. Neurosurgery 1988;23:545-56.  Back to cited text no. 1
    
2.Daumas-Duport C, Varlet P, Bacha S, Beuvon F, Cervera-Pierot P, Chodkiewicz JP. Dysembryoplastic neuroepithelial tumor: Nonspecific histological forms:A study of 40 cases. J Neurooncol 1999;41:267-80.  Back to cited text no. 2
    
3.Hammond RR, Duggal N, Woulfe JM, Girvin JP. Malignant transformation of a dysembryoplastic neuroepithelial tumor. Case report. J Neurosurg 2000;92:722-5.  Back to cited text no. 3
    
4.Josan V, Smith P, Kornberg A, Rickert C, Maixner W. Development of a pilocytic astrocytoma in a dysembryoplastic neuroepithelial tumor. Case report. J Neurosurg 2007;106:509-12.  Back to cited text no. 4
    
5.5 Rushing EJ, Thompson LD, Mena H. Malignant transformation of a dysembryoplastic neuroepithelial tumor after radiation and chemotherapy. Ann Diagn Pathol 2003;7:240-4.  Back to cited text no. 5
    


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