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