gms | German Medical Science

65th Annual Meeting of the German Society of Neurosurgery (DGNC)

German Society of Neurosurgery (DGNC)

11 - 14 May 2014, Dresden

Lhermitte-Duclos disease / dysplastic gangliocytoma of the cerebellum – a report of four cases

Meeting Abstract

  • Jana Kohl - Klinik für Neurochirurgie, Universitätsklinikum Magdeburg
  • Christian Mawrin - Institut für Neuropathologie, Universitätsklinikum Magdeburg
  • Boris A. Jöllenbeck - Klinik für Neurochirurgie, Universitätsklinikum Magdeburg
  • Imre Bondar - Klinik für Neurochirurgie, Universitätsklinikum Magdeburg
  • Cordula Scherlach - Institut für Neuroradiologie, Universitätsklinikum Magdeburg
  • Thomas Schneider - Klinik für Neurochirurgie, Universitätsklinikum Magdeburg

Deutsche Gesellschaft für Neurochirurgie. 65. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC). Dresden, 11.-14.05.2014. Düsseldorf: German Medical Science GMS Publishing House; 2014. DocP 018

doi: 10.3205/14dgnc413, urn:nbn:de:0183-14dgnc4137

Published: May 13, 2014

© 2014 Kohl et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.



Objective: Dysplastic gangliocytoma is a rare lesion of the cerebellum. It remains unclear whether to speak of a hamartoma or a tumor. It’s a slowly growing lesion associated with a mutation of the PTEN gene. PTEN is a tumor suppressor gene located at chromosome 10. The encoded protein acts as a phosphatase which is part of a chemical pathway that signals cells to undergo programmed cell death (apoptosis). The dysplastic gangliocytoma of the cerebellum is associated with the Cowden disease, which is a rare autosomal dominant phacomatosis characterized by multiple hamartomas like intestinal polyposis and by a high incidence of malignant tumors, especially cancers of the breast, the thyroid and the uterus.

Method: We report four cases of patients treated between 2008 to 2011. Two of patients were female. Median age at the time of surgery was 50.

Results: The first patient presented with an epileptic seizure and headaches. The following MRI-scan demonstrated a right temporal tumor and a giant lesion in the right cerebellar hemisphere. The temporal tumor which should be responsible for the leading symptom presented as meningioma WHO grade I. Additionally, hepatic lesions were diagnosed by ultrasound and CT scans. Patient's history showed an additional lesion of thyroid seeming to be an adenoma. The patient had to undergo psychiatric treatment because of depression later on. The second patient presented with schizophrenia. In the context of screening examinations a cerebellar lesion was diagnosed by MRI. The third patient has reported about a progredient gait disturbance for 5 years before diagnosis. The fourth patient suffered from dizziness and headache. The patient underwent resection of colorectal polyps one year before diagnosis of gangliocytoma. The therapy consisted of resection of the cerebellar “tumor” mass in all cases. The diagnosis of a dysplastic gangliocytoma of the cerebellum was confirmed by histological analysis. Three patients underwent follow-up by MRI. Two patients showed no recurrence of the lesion about 2, respectively 5 years, one patient showed a non-growing lesion about 4,5 years. One patient was lost to follow-up.

Conclusions: All patients presented symptoms years before diagnosis. Due to the association of Cowden disease with increased incidence of various malignant tumors, a close follow-up is mandatory in patients diagnosed with Lhermitte-Duclos disease.