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59. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC)
3. Joint Meeting mit der Italienischen Gesellschaft für Neurochirurgie (SINch)

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

01. - 04.06.2008, Würzburg

Cerebellar medulloblastoma associated with pineal enhancing mass lesion: case report and review of the literature

Meeting Abstract

  • corresponding author G. Bertani - U.O. Neurochirurgia, Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milano
  • M. Caroli - U.O. Neurochirurgia, Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milano
  • M. A. S. Zella - U.O. Neurochirurgia, Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milano
  • P. Rampini - U.O. Neurochirurgia, Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milano
  • D. Bauer - Anatomia Patologica, Ospedale San Paolo, Milano, Italy
  • S. M. Gaini - U.O. Neurochirurgia, Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milano

Deutsche Gesellschaft für Neurochirurgie. Società Italiana di Neurochirurgia. 59. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC), 3. Joint Meeting mit der Italienischen Gesellschaft für Neurochirurgie (SINch). Würzburg, 01.-04.06.2008. Düsseldorf: German Medical Science GMS Publishing House; 2008. DocP 034

Die elektronische Version dieses Artikels ist vollständig und ist verfügbar unter: http://www.egms.de/de/meetings/dgnc2008/08dgnc302.shtml

Veröffentlicht: 30. Mai 2008

© 2008 Bertani et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.de). Er darf vervielf&aauml;ltigt, verbreitet und &oauml;ffentlich zug&aauml;nglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.


Gliederung

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Objective: To report an unusual case of cerebellar medulloblastoma associated with pineal enhancing mass lesion, and a review of the literature.

Case report: Medulloblastoma is a malignant and invasive embryonal tumour of the cerebellum (WHO grade IV), and it is the most frequent type of malignant brain tumour in childhood (0 to 18 years), accounting for 17,2% of these tumours. This kind of tumour has a high proclivity for metastasis within the CNS, along CSF pathways: it is estimated that in 20–25% of patients affected by medulloblastoma, CSF dissemination is present, especially in the spinal cord. The occurrence of metastatic disease is an important poor prognostic factor: these patients are categorized as high risk and, after surgery, they are treated with aggressive chemo- and radiation therapies (5-years survival is about 40%, while in standard risk patients 5-years survival is 70%).

Here we present the case of a twelve years-old girl, who presented with a 4 months history of headache, nausea, vomiting and weight loss, associated with apathy and a drop in scholastic progress. Neurological exam showed only mild dysmetria. She was admitted to the pediatric emergency department where she performed head CT and MR imaging which showed a vermian mass lesion growing into the fourth ventricle: the lesion was hyperdense on CT, dyshomogeneous on MR, with some small cysts. This lesion was enhancing, not homogeneously, after contrast medium administration. A second mass lesion was present in the pineal region, partly enhancing and partly cystic as well, with a neuroradiological suggestion of metastatic medulloblastoma. Spinal MRI was negative. The patient was transferred to the Department of Neurosurgery; a suboccipital craniotomy was performed, with removal of the posterior fossa lesion, and, in the same surgery, of the pineal lesion by an infratentorial-supracerebellar approach. At postoperative MRI there was no residual tumour. The histological exam was performed by our neuropathologists, and then the case was reviewed by an expert, independent neuropathologist: they reported that the cerebellar lesion was indeed an undifferentiated medulloblastoma, while the pineal lesion was consistent with pineal cyst, which is a benign lesion.

Conclusions: Pineal cysts are considered anatomic variants, usually asimptomatic. In this patient, careful staging allowed for correct risk categorization and consequent adjuvant treatment. A review of the literature is also presented.