Dynamic changes in magnetic resonance imaging appearance of dysembryoplastic neuroepithelial tumor with or without malignant transformation

Report of 2 cases

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Dysembryoplastic neuroepithelial tumors (DNETs) have conventionally been regarded as benign and stable tumors and considered curable with surgery without adjunctive therapy. Recently, recurrent DNETs with or without malignant transformation have been described. The authors report 2 unusual cases of DNET: 1) an enlarging lesion that developed an enhancing component over the natural course of 4 years, and 2) a recurrent DNET that developed an enhancing component 10–11 years after gross-total resection. The patient in the first case was treated with subtotal resection and adjuvant radiochemotherapy; histological examination of the tumor led to the diagnosis of DNET, WHO Grade I, for the nonenhancing component and anaplastic oligodendroglioma, WHO Grade III, for the enhancing component. The patient in the second case was treated with repeat gross-total resection; the original tumor had been histologically diagnosed as DNET, and the nonenhancing and enhancing components of the recurrent tumor were diagnosed as simple and complex forms of DNET, respectively. These and previous reports suggest an aggressive subtype of DNETs. If follow-up MRI reveals progressive behavior, resection should be performed without delay. Additional radiochemotherapy is needed if the histological diagnosis demonstrates malignant transformation.

Abbreviations used in this paper:CDKN2A = cyclin-dependent kinase inhibitor 2A; DNET = dysembryoplastic neuroepithelial tumor; EGFR = epidermal growth factor receptor; ERBB2 = v-erbb2 erythroblastic leukemia viral oncogene homolog 2; GFAP = glial fibrillary acidic protein; IDH1/2 = isocitrate dehydrogenase 1 and 2; NFP = neurofilament protein; Olig2 = oligodendrocyte transcription factor 2; PTEN = phosphatase and tensin homolog; SYN = synaptophysin; TP53 = tumor protein p53; WHO = World Health Organization.

Article Information

Address correspondence to: Toshihiro Kumabe, M.D., Ph.D., Department of Neurosurgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-cho, Aoba-ku, Sendai-shi, Miyagi 980-8574, Japan. email: kuma@nsg.med.tohoku.ac.jp.

Please include this information when citing this paper: published online February 22, 2013; DOI: 10.3171/2013.1.PEDS11449.

© AANS, except where prohibited by US copyright law.

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    Case 1. Axial CT and MR images obtained 10 months after birth (A–C) and at age 4 years (D–F) and postoperative follow-up MR image (G). A: CT scan showing a slightly hypodense lesion in the right hemisphere (arrow). B: T1-weighted MR image obtained after Gd administration demonstrating no enhancement. C: FLAIR MR image disclosing a hyperintense lesion predominantly in the right postcentral gyrus (arrow), with invasion into the right precentral knob (arrowhead). D: CT scan clarifying marked enlargement of the hypodense lesion, with an emerging hyperdense ringlike nodule inside the lesion (arrow). E: T1-weighted MR image obtained after Gd administration showing ringlike enhancement (arrow) inside the hyperintense rim on the T1-weighted MR image obtained without Gd (not shown). F: T2-weighted MR image depicting a hyperintense lesion predominantly in the right postcentral gyrus corresponding to the hypointense lesion on the T1-weighted MR image. The hyperintense rim on the T1-weighted MR image appears as hypointense on the T2-weighted MR image (arrow). G: Postoperative axial T1-weighted MR image obtained after Gd administration demonstrating no enhancing lesion.

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    Case 1. Photomicrographs of the surgical specimen from the lesion that showed no Gd enhancement on MRI. A: Multiple cystic structures are observed on a mucinous background. H & E, original magnification × 100. B: High-power view of A, showing floating neurons (arrows) scattered in the cystic structure. H & E, original magnification × 400. C–E: Immunostaining for GFAP (C), Olig2 (D), and NFP (E) are all positive. Original magnification × 100. F: The Ki 67 labeling index was 2%. Original magnification × 100.

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    Case 1. Photomicrographs of the surgical specimen from the lesion that showed enhancement on MRI. A: Markedly proliferated atypical glial cells and chicken-wire appearances are observed. H & E, original magnification × 100. B: High-power view of A, showing microvascular proliferation. H & E stain, original magnification × 400. C–E: Immunostaining for GFAP (C) and NFP (E) are negative, but Olig2 (D) is strongly positive. Original magnification × 100. F: The Ki 67 labeling index was focally up to 30%. Original magnification × 100.

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    Case 2. Axial T1-weighted MR images obtained after Gd administration before (A) and after (B) the first operation (at age 3 years) and before (C) and after (D) the second operation (at age 14 years). A: Preoperative image showing a hypointense nonenhancing lesion in the right facial motor area. B: Postoperative image showing total resection. C: Imaging performed 11 years later revealed a hypointense lesion with an enhancing area (arrow) that was not evident on earlier studies. D: Postoperative image showing no enhancing lesion.

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    Case 2. Photomicrographs of the second surgical specimen from the nonenhancing lesion in Fig. 4C. A: Glioneuronal elements in a columnar orientation are observed on a mucinous background. H & E, original magnification × 100. B: High-power view of A, showing floating neurons (arrow) scattered in the cystic structure. H & E stain, original magnification × 400. C–E: Immunostaining for GFAP (C), Olig2 (D), and NFP (E) were all positive. Original magnification × 400. F: The Ki 67 labeling index was 2%. Original magnification × 400.

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    Case 2. Photomicrographs of the second surgical specimen from the enhancing lesion. A: Oligodendroglia-like cells, and partly microvascular proliferation, are found with no apparent endothelial swelling. H & E stain, original magnification × 100. B: High-power view of A, showing no endothelial swelling and proliferation. H & E stain, original magnification × 400. C–E: Immunostaining for GFAP (C) and Olig2 (D) are positive, but NFP (E) is negative. Original magnification × 400. F: The Ki 67 labeling index was 2%. Original magnification × 400.

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