|Case of the Week - Patient Summary 14113|
Peer Reviewed and Certified -
|Demographics: 25 y.o. man|
|History & Chief complaint:|
| 25 year old male with uncontrolled seizures |
|Physical exam and Laboratory:|
| Non-contributory |
|Summary of Findings:|
|2 cm lesion left parietal lobe. No edema or contrast enchancment.|
| Dysembryoplastic neuroepithelial Tumor, DNT, DNET |
|Confirmed by: Surgery and pathology|
|Treatment and Followup:|
|Surgical resection and continued antiseizure medication.|
|Neoplasm Name: Dysembryoplastic neuroepithelial Tumor, DNT, DNET
1: Epilepsia 1993 Jul-Aug;34(4):609-15
Coexistence of neoplasia and cortical dysplasia in patients presenting with
Prayson RA, Estes ML, Morris HH.
Department of Anatomic Pathology, Cleveland Clinic Foundation, Ohio 44195-5138.
Tumors and cortical dysplasia are associated with epilepsy, but few studies have examined the coexistence of neoplasia and dysplasia in these patients. We studied 13 patients (age 4-29 years) with recurrent seizures of 1 month to 21-year' duration (median 72 months). Ten patients were aged < 21 years. Imaging studies localized the lesion to the temporal lobe (10 patients), parietal lobe (2 patients), and frontal lobe (1 patient). Tumors included ganglioglioma (8 patients), dysembryoplastic neuroepithelial tumor (DNT) (3 patients), and low-grade astrocytoma (2 patients). Cortical dysplasia, including atypical aggregates of neurons (6 patients), multifocal loss of the cortical laminar architecture (7 patients), and neurons in the molecular layer of the cortex (3 patients) were observed near but separate from the tumor. Coexistence of certain tumors with cortical dysplasia, most frequently observed in the pediatric population, suggests a hamartomatous/dysplastic nature of the neoplasms.
PMID: 8330569 [PubMed - indexed for MEDLINE]
2: Hum Pathol 1995 May;26(5):493-500
Cortical dysplasia: a histopathologic study of 52 cases of partial lobectomy in patients with epilepsy.
Prayson RA, Estes ML.
Department of Anatomic Pathology, Cleveland Clinic Foundation, OH 44195-5138, USA.
In utero migrational abnormalities account for most cases of cortical dysplasia. The histopathologic appearance of cortical dysplasia is often varied, making recognition and classification difficult. We studied 52 patients with cortical dysplasia who underwent partial lobectomy for medically intractable seizures in order to devise a simple histopathologic classification schema. The incidence of observed dysplasia in lobectomy specimens over an 11-year period (n = 360) was 14%. Patients ranged in age from 3 months to 47 years at the time of surgery (mean, 19 years; 29 male and 23 female patients). The temporal lobe was involved in 34 patients, frontal lobe in 18, parietal lobe in four, and occipital lobe in three. In three patients multiple lobes showed dysplasia. Dysplasia was right-sided in 29 patients and left-sided in 23 patients. Dysplasia was focal in 23 patients, multifocal in four patients, and diffuse in 25 patients. Three main histologic patterns of cortical dysplasia were observed: (1) a cortical laminar architectural disorganization and/or malalignment of neurons (26 patients), (2) clusters of atypical neurons and glia within the cortex (28 patients), and (3) a hypercellular molecular layer with increased numbers of neurons and glia (31 patients). In 23 patients more than one pattern of dysplasia was identified. Coexistent tumors were present in 13 patients, including ganglioglioma (eight patients), dysembryoplastic neuroepithelial tumor (three patients), and low-grade astrocytoma (two patients). Tuberous sclerosis was present in four patients. We conclude that most types of cortical dysplasia can be divided into three main histologic patterns, facilitating the recognition of dysplasia. In addition to the known association with tuberous sclerosis, tumors may coexist with cortical dysplasia.
PMID: 7750933 [PubMed - indexed for MEDLINE]
3: AJNR Am J Neuroradiol 1996 Mar;17(3):419-30
Comment in: AJNR Am J Neuroradiol. 1997 Sep;18(8):1592.
Dysembryoplastic neuroepithelial tumors: MR and CT evaluation.
Ostertun B, Wolf HK, Campos MG, Matus C, Solymosi L, Elger CE, Schramm J, Schild HH.
Department of Neuroradiology/Radiology, University of Bonn, Germany.
PURPOSE: To evaluate dysembryoplastic neuroepithelial tumors (DNTs) on MR and CT studies and to compare DNT with oth ...
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