Dysembryoplastic neuroepithelial tumor
| Classification according to ICD-10 | |
|---|---|
| D33 | Benign neoplasm of the brain and other parts of the central nervous system ICD-O 9413/0 |
| ICD-10 online (WHO version 2019) | |
The dysembryoplastic neuroectodermal tumor (abbreviated DNET or DNT ) is a rare, benign brain tumor that was first described by Daumas-Duport in 1988. These tumors occur mainly in children, adolescents and young adults and manifest themselves in epileptic seizures , some with a history of many years. The tumors are usually located superficially in the cerebrum , but infratentorial localizations (cerebellum) have also been described. Apart from epileptic seizures, which are difficult to treat, and occasionally headaches, they do not lead to any further symptoms. The prognosis is favorable, which is why DNETs are classified as grade I according to the WHO classification of tumors of the central nervous system .
Epidemiology
The incidence (frequency) of DNET is difficult to estimate because several reports have come from specialized centers specializing in the treatment of epilepsy. It is estimated that around 1.2% of neuroepithelial tumors meet the criteria of DNET. Patients are usually young, most of them children. Initial diagnosis of DNET is rare in adults.
Diagnosis
Imaging
Magnetic resonance imaging (MRI, MRI) or computed tomography (CT) imaging shows lesions in the cerebral cortex. The lesions often extend the full thickness of the cortex.
On the CT one sees hypodense, well-demarcated cerebral cortex lesions. Cysts are often found, calcifications less common. Enrichment of contrast media is not typical. There is no space-occupying displacement of the surrounding tissue. Sometimes the skullcap is deformed over the tumor.
The MRI shows the lesions to be well demarcated, not occupying space and without perifocal edema (fluid absorption in the surrounding brain parenchyma). The multinodular configuration (consisting of many node-like structures) is described as typical. Here, too, there is no contrast enhancement in the majority of cases.
pathology
Macroscopically, the tumors are soft and gelatinous. The tumors can form several nodes in the cerebral cortex and occasionally also extend into the medullary bed (white brain matter). The most frequently affected brain region is the temporal lobe (temple lobe), followed by the frontal lobe (frontal lobe) and less often the parietal lobe (parietal lobe).
Microscopically, the most essential feature is a complex of structures consisting of glial and neural elements ( glioneuronal elements ). The glial elements are strands of glial fibers and vessels. They delimit “slime lakes” in which there are largely mature neurons.
Differential diagnosis
The most important differential diagnoses of DNET are oligodendrogliomas and gangliogliomas .
Therapy and prognosis
Treatment consists of surgical removal of the tumor, which usually also enables seizure control. Dysembryoplastic neuroectodermal tumors probably do not have an increased tendency to malignant degeneration, and even if only partially removed they do not show any progressive growth.
swell
- WHO Classification of Tumors of the Central Nervous system. IARC Press Lyon 2007.
Individual evidence
- ↑ C. Daumas-Duport et al .: Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases. In: Neurosurgery . (1988) 23 (5): 545-56. PMID 3143922 .
- ↑ a b W. Reiche et al .: Dysembryoplastic neuroepithelial tumor (DNT). Pattern of neuroradiological findings. Radiologist. (1996) 36 (11): 884-9. PMID 9036430 .
- ↑ R. Stanescu Cosson et al .: Dysembryoplastic neuroepithelial tumors: CT, MR imaging findings and follow-up: a study of 53 cases. J Neuroradiol. (2001) 28 (4): 230-40. PMID 11924137 .
