Ganglioglioma

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Classification according to ICD-10
D33 Degeneration of ganglion cells in the brain
ICD-O 9505/1
ICD-10 online (WHO version 2019)

The ganglioglioma is a slowly growing tumor inherent in the brain, which partly contains neuronal but also glial cells and thus belongs to the mixed neuronal / glial tumors. According to the WHO classification of tumors of the central nervous system , gangliogliomas are classified as grade I or grade II (histologically benign tumors).

Symptoms

Due to their cortical location, gangliogliomas often cause epilepsy and are the most common tumor associated with chronic temporal lobe epilepsy. In general, the clinical appearance is characterized by chronic, drug-resistant seizures, which are often diagnosed late as a tumor.

Frequency and location

Gangliogliomas are very rare and make up less than 2% of the brain's own tumors. In principle, they can occur in any age group, but preferably in young adulthood (between around 9 and 25 years of age). In contrast, it is the most common tumor type in patients with chronic, refractory focal epilepsy.

Histologically, this well-differentiated neuroepithal tumor is characterized by neoplastic ganglion cells and neoplastic glial cells - they have a higher proportion of glial cells than gangliocytoma. In principle, they can occur anywhere in the central nervous system , although around two thirds of them are localized in the area of ​​the temporal lobe .

Differential diagnosis

Differential diagnoses include dysembryoplastic neuroepithelial tumor (DNT), pleomorphic xanthoastrocytoma , and pilocytic astrocytoma , which also tend to occur in adolescence.

therapy

The therapeutic approach is surgical resection (removal) of the tumor; symptomatic gangliogliomas are removed . If the resection is incomplete, years after the operation are possible; Recurrences (recurrence) do occur.

Regular clinical epileptological follow-up with documentation of the seizure situation and regular magnetic resonance imaging controls are required.

Anaplastic ganglioglioma

Classification according to ICD-10
D33 Degeneration of ganglion cells in the brain
ICD-O 9505/3
ICD-10 online (WHO version 2019)

The anaplastic ganglioglioma is a malignant (glial) variant of the ganglioglioma and is characterized by a lack of differentiation. These usually arise from previously low-grade tumors (WHO grade III). They are also very rare among gangliogliomas (around 3–5% of all gangliogliomas). The recurrence rate with this form is much higher.

The most severe form, the dedifferentiated ganglioglioma, has WHO grade IV.

Desmoplastic infantile ganglioglioma

Classification according to ICD-10
D33 rare variant of mixed neural-glial tumors
ICD-10 online (WHO version 2019)

Because of its characteristic clinical and neuropathological features, this rare tumor was included in the WHO list (1993); Grade I. This tumor occurs predominantly in the first two years of life and is located in the cerebral hemispheres.

Macrocephaly and / or complex focal seizures can be seen in infancy .

Individual evidence

  1. gliomas. on the website of the Clinic and Polyclinic for Neurosurgery at the University Medical Center Greifswald
  2. Ganglioglioma of the brain WHO grade 1-2. ( Memento of the original from March 9, 2016 in the Internet Archive ) Info: The archive link was inserted automatically and has not yet been checked. Please check the original and archive link according to the instructions and then remove this notice. Definition at medrapid.info @1@ 2Template: Webachiv / IABot / www.medrapid.info
  3. ^ A b Uwe Schlegel, Michael Weller, Manfred Westphal: Neuroonkologie . 2., revised. u. exp. Edition. Thieme, Stuttgart 2003, ISBN 3-13-109062-6 , pp. 28/29.
  4. a b Thomas J. Vogl, Wolfgang Reith, Ernst J. Rummeny: Diagnostic and interventional radiology. 1st edition. Springer, Berlin / Heidelberg 2010, ISBN 978-3-540-87667-0 , pp. 166/167.
  5. Peter Berlit: Clinical Neurology . 3rd, exp. and completely revised Edition. Springer, Berlin / Heidelberg 2011, ISBN 978-3-642-16919-9 , p. 702.
  6. Peter Berlit: Therapielexikon Neurologie. 1st edition. Springer, Berlin / Heidelberg 2004, ISBN 3-540-67137-4 , pp. 482/483.
  7. ^ Uwe Schlegel, Michael Weller, Manfred Westphal: Neuroonkologie . 2., revised. u. exp. Edition. Thieme, Stuttgart 2003, ISBN 3-13-109062-6 , p. 226.
  8. Dieter-Karsten Böker, H.-D. Mennel, Paul Hermanek: Classification of malignant tumors of the CNS and the eyes. 1st edition. Springer, Berlin 2002, ISBN 3-540-42620-5 , pp. 31/32.
  9. S. Hahn, W. Schmiegel, Detlev Ganten, Klaus Ruckpaul: Molecular medical principles of non-hereditary tumor diseases. 1st edition. Springer, 2002, ISBN 3-540-41577-7 , p. 16.

literature

Web links