ManuscriptDysembryoplastic neuroepithelial tumors (DNET) are usually benign lesions that arise in cortical regions and are discovered after new onset of seizure. These lesions have many different imaging characteristics. We report a patient with a presumed low-grade medial temporal lobe lesion that was followed for many years without any change in size or imaging characteristics. This previously non-enhancing tumor evolved to become contrast enhanced on routine imaging without apparent tumor growth. The patient underwent surgery, and the pathology was confirmed as a DNET with no atypical  changes. This case demonstrates the potential that DNETs may exhibit a changing MRI pattern over time. Natural history, imaging characteristics, and management are reviewed

Caamano, Eryn

Couldwell, William T.

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The University of Utah: J. Willard Marriott Digital Library

c c c c University of Utah Institutional Repository Author Manuscript Jensen et al. 1 Development of Contrast Enhancement after Long-Term Observation of a Dysembryoplastic Neuroepithelial Tumor Randy L. Jensen M.D., Ph.D., Eryn Caamano M.D., Elizabeth M. Jensen M.D., William T. Couldwell M.D. Ph.D, Department of Neurosurgery, University of Utah, Salt Lake City, Utah 84132 (EC, WTC, RLJ) and Huntsman Cancer Institute, University of Utah, Salt Lake City, Utah 84112 (WTC, RLJ) and Department of Pathology, Salt Lake Veterans Health Care System, Salt Lake City, Utah 84148 (EMJ) Corresponding AuthorlReprint Requests: Randy Jensen, M.D., Ph.D. Department of Neurosurgery University of Utah 30 North 1900 East, 3B-409 SOM Salt Lake City, Utah 84132-2303 Telephone 801-581-6908 Fax 801-581-4385 Email randy.jensen@hsc.utah.edu Running Head: New-onset contrast-enhancing DNET c c c c Abstract University of Utah Institutional Repository Author Manuscript Jensen et al. 2 Dysembryoplastic neuroepithelial tumors (DNET) are usually benign lesions that arise in cortical regions and are discovered after new onset of seizure. These lesions have many different imaging characteristics. We report a patient with a presumed low-grade medial temporal lobe lesion that was followed for many years without any change in size or imaging characteristics. This previously non-enhancing tumor evolved to become contrast enhanced on routine imaging without apparent tumor growth. The patient underwent surgery, and the pathology was confirmed as a DNET with no atypical changes. This case demonstrates the potential that DNETs may exhibit a changing MRI pattern over time. Natural history, imaging characteristics, and management are reviewed. Key words: Brain tumor; DNET; imaging; seizures c c c c Introduction University of Utah Institutional Repository Author Manuscript Jensen et al. 3 Dysembryoplastic neuroepithelial tumors (DNET) are thought to arise during embryogenesis. They are usually clinically benign, stable lesions that arise in cortical regions, most frequently in the temporal lobe. Clinically, they present with partial epilepsy that is often drug resistant, usually before the age of 20 [1-3]. A wide variety of imaging techniques have been used with variable success to predict the histology preoperatively. Contrast enhancement has been demonstrated in one third of cases [3]. We report a patient with a presumed low-grade medial temporal lobe lesion that was followed for many years without any change in size or imaging characteristics. This previously non-enhancing tumor evolved to become contrast enhanced on routine imaging. The patient underwent surgery and the pathology was confirmed as a DNET with no atypical changes. This case demonstrates the potential that DNETs may exhibit a changing MRI pattern over time and that the development of contrast enhancement does not necessarily imply malignant progression. Case Report Clinical Presentation and Physical Exam The patient is a right-handed woman with a history of partial complex seizures since the age of 25. An MRI four years later showed a low-intensity lesion in the left mesiotemporallobe that involved the uncus (Figure 1). Various options were discussed with the patient, but she was resistant to proceed with surgical therapy. The patient's seizures were initially treated medically with limited success and a vagal nerve stimulator was placed with little improvement. She remained medically intractable (3 medications) c c c c University of Utah Institutional Repository Author Manuscript Jensen et al. 4 for several years. In the year preceding her eventual surgery, the patient's seizures grew progressively worse despite different medication trials. On MRI, which had remained stable over 15 years (the patient was now 46 years of age), the size of the mass and fluid attenuated inversion recovery (FLAIR) signal were unchanged, but new enhancement of the lesion that was concerning for progression of the disease was apparent (Figure 2). MR spectroscopy showed decreased N-acetyl aspartate (NAA) and increased choline consistent with tumor as opposed to cortical dysplasia (data not shown). The patient underwent an intra-arterial sodium amy tal (Wada) test, which demonstrated that the left hemisphere was dominant for language functioning and the right hemisphere could support memory. At the time of surgery, the patient was having between 2 and 11 seizures in a single week. The patient's physical and neurology exams were intact. Surgery At the time of surgery, intraoperative electrocorticography demonstrated active spiking over the medial temporal structures. A limited lateral neocortical resection was performed superiorly (2 cm of superior temporal gyrus), extending to include 4 cm of the inferior temporal gyrus. The amygdala and anterior aspect of the hippocampus back to the posterior margin of the cerebral peduncle were resected; the anterior aspect was actively involved with the tumor. The histology of the tumor was consistent with DNET, showing predominantly small, round cells and scattered neurons (Figure 3). The neurons had prominent nucleoli and were immunohistochemically positively for synaptophysin. The majority of the small round cells were consistent with astrocytes and positive for glial fibrillary acidic protein (GFAP). The MIB-l marker demonstrated only a rare c c c c University of Utah Institutional Repository Author Manuscript Jensen et al. 5 positive nucleus. Postoperative imaging demonstrated complete resection of the tumor (Figure 4). The patient had an uneventful postoperative course and remains seizure free 6 months postoperatively. Anti-epileptic medications have been discontinued. Discussion Clinical presentation and histology Dysembryoplastic neuroepithelial tumors occur predominately in the temporal lobe and present with focal epilepsy in young individuals. They are usually benign supratentorial tumors that contain both neuronal and glial components. Although these tumors generally have a benign course, one DNET with malignant transformation has been reported [4]. In the WHO classification, DNETs are included in the category of neuronal and mixed neuroglial tumors [1]. A diagnosis of DNET should be postulated if there is presence of partial seizures, absence of a neurological deficit, and the presence of a cortical lesion without peritumoral edema [2]. Histologically, DNETs are composed of a heterogeneous collection of oligodendrocyte-like cells with mature ganglion cells and astrocytes that can be surrounded by a myxoid or mucinous matrix [3]. Three histologic forms are recognized. The association of a specific glioneuronal element (SGNE) with glial nodules and multinodular architecture characterizes the complex form. The simple form only has the SGNE. The third form is a nonspecific form that does not display a SGNE but displays the same clinical and imaging features as a complex DNET [4] . Radiological appearance c c c c University of Utah Institutional Repository Author Manuscript Jensen et al. 6 Although often variable and nonspecific, neuroimaging can be useful in the diagnosis of DNET. These tumors appear as hypointense cyst-like lesions on CT with a triangular pattern of distribution and occasion overlying skull deformation [4]. Peri tumoral edema and mass effect are usually not found. They tend to be hypointense on Tl-weighted imaging on MRI and hyperintense on T2-weighted imaging [3,4]. As mentioned above, contrast enhancement has been shown to be present in one third of cases, however no change has been reported in serially followed tumors without obvious tumor growth [3]. Various imaging techniques, including magnetic resonance spectroscopy (MRS), diffusion weighted MRI (DWMRI), and positron emission tomography (PET), have been used to distinguish DNETs from other common brain neoplasms with limited success [5,6]. DNETs can have close to normal spectra on MRS and high apparent diffusion coefficient (ADC) values on DWMRI [5]. Radiographic behavior in conservatively managed cases In cases where observation has been used to manage the patient conservatively, tumor size has been monitored with CT or MR studies. Ostertun et al. [7] followed six patients with serial CT or MR studies obtained over a 13-year period, two of whom demonstrated slow progressive growth [3]. In one patient, the tumor showed calcification with progressive mass effect and pre-Iesional edema. In the other patient, MR studies showed growth of the cystic tumor parts with extension into the lower insular cortex and deep into the white matter. New tumor enhancement was demonstrated in this second patient but only with increased growth of the lesion [3]. However, the patient in the present report appears to be unique in that no cases have been reported in the current c c c c University of Utah Institutional Repository Author Manuscript Jensen et al. 7 literature of a previously non-enhancing lesion developing enhancement without tumor growth. As mentioned, our patient showed a low-density lesion on CT in the left mesiotemporallobe that involved the uncus. On MRI, the patient had a stable, non-enhancing low-intensity Tl-weighted cystic lesion in the left temporal lobe. With the worsening of the patient's seizures and failure of medication trials, surgical resection of the tumor was finally considered. Preoperative imaging demonstrated that the left medial temporal mass had new enhancement with unchanged size and FLAIR signal compared with prior imaging. The mass had been followed for 15 years with no changes until the time of surgery. Histology of the specimen obtained from the mesiotemporal gray and white matter was consistent with a benign DNET. The interesting possibility exists that the new enhancement may relate to the increased seizure activity manifest in this patient, similar to enhancement seen with non-neoplastic foci of epilepsy. This case demonstrates the potential for changing MRI enhancement pattern in an otherwise stable benign DNET. Acknowledgments We thank Kristin Kraus for her editorial assistance in preparing this paper. c c c c University of Utah Institutional Repository Author Manuscript Jensen et al. 8 References 1. Kleihues P, Cavenee WK (eds): World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of the Nervous System. Lyon, IARC Press, 2000 2. Daumas-Duport C: Dysembryoplastic neuroepithelial tumours. Brain Pathol 3:283-295, 1993 3. Shin JH, Lee HK, Khang SK, Kim DW, Jeong AK, Ahn KJ, Choi CG, Suh DC: Neuronal tumors of the central nervous system: radiologic findings and pathologic correlation. Radiographics 22: 1177 -1189, 2002 4. Fernandez C, Girard N, Paz Paredes A, Bouvier-Labit C, Lena G, Figarella-Branger D: The usefulness of MR imaging in the diagnosis of dysembryoplastic neuroepithelial tumor in children: a study of 14 cases. AJNR Am J Neuroradiol 24:829-834, 2003 5. Bulakbasi N, Kocaoglu M, Ors F, Tayfun C, Ucoz T: Combination of single-voxel proton MR spectroscopy and apparent diffusion coefficient calculation in the evaluation of common brain tumors. AJNR Am J Neuroradiol 24:225-233, 2003 6. Maehara T, Nariai T, Arai N, Kawai K, Shimizu H, Ishii K, Ishiwata K, Ohno K: Usefulness of [llC]methionine PET in the diagnosis of dysembryoplastic neuroepithelial tumor with temporal lobe epilepsy. Epilepsia 45:41-45,2004 7. Ostertun B, Wolf HK, Campos MG, Matus C, Solymosi L, Elger CE, Schramm J, Schild HH: Dysembryoplastic neuroepithelial tumors: MR and CT evaluation. AJNR Am J NeuroradioI17:419-430, 1996 c c c c University of Utah Institutional Repository Author Manuscript Jensen et al. 9 Figure 1. a. Axial Tl-weighted magnetic resonance image demonstrating a left mesial low-attenuated lesion. b. Axial Tl-weighted magnetic resonance image after gadolinium administration. c. Coronal Tl-weighted magnetic resonance image after gadolinium administration. These images demonstrate a non-enhancing 2.1 x 2.1 x 2.0 cm mass with Tl hypointensity involving the left temporal lobe uncus, which abuts but does not significantly compress the adjacent left cerebral peduncle. Figure 2. a. Axial Tl-weighted magnetic resonance image demonstrating a left mesial low-attenuated lesion. b. Axial Tl-weighted magnetic resonance image after gadolinium administration. c. Coronal Tl-weighted magnetic resonance image after gadolinium administration. In these images, which were obtained 15 years after those in Figure 1, the Tl-weighted hypointense lesion measures 2.3 x 2.2 x 2.2 cm. A new enhancing focus at c c c c University of Utah Institutional Repository Author Manuscript Jensen et al. 10 the center of the lesions measures 4 mm x 8 mm and suggested progression of the disease. Figure 3. Hematoxylin- and eosin-stained section demonstrating small, round cells, set in a cystic background filled with pale lavender fluid. A few neurons containing prominent nucleoli float in the fluid. The synaptophysin and glial fibrillary acidic protein stains were immunohistochemically positive (not shown). c c c c University of Utah Institutional Repository Author Manuscript Jensen et al. 11 Figure 4. a. Axial Tl-weighted image after gadolinium administration. b. Coronal Tl-weighted image after gadolinium administration. These postoperative images demonstrate complete resection of the tumor. 

Development of contrast enhancement after long-term observation of a dysembryoplastic neuroepithelial tumor

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Development of contrast enhancement after long-term observation of a dysembryoplastic neuroepithelial tumor

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