gms | German Medical Science

60th Annual Meeting of the German Society of Neurosurgery (DGNC)
Joint Meeting with the Benelux countries and Bulgaria

German Society of Neurosurgery (DGNC)

24 - 27 May 2009, Münster

Neurosurgical management of Brown-Sequard syndrome and tetraplegia of a 4-year-old girl caused by an atypical teratoid rhabdoid tumor C5/6/7

Meeting Abstract

Search Medline for

  • A. Niedermeier - Klinik für Neurochirurgie, Universitätsklinikum Ulm
  • D. Woischneck - Klinik für Neurochirurgie, Universitätsklinikum Ulm

Deutsche Gesellschaft für Neurochirurgie. 60. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit den Benelux-Ländern und Bulgarien. Münster, 24.-27.05.2009. Düsseldorf: German Medical Science GMS Publishing House; 2009. DocP08-10

DOI: 10.3205/09dgnc338, URN: urn:nbn:de:0183-09dgnc3385

Published: May 20, 2009

© 2009 Niedermeier et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.


Outline

Text

Objective: Atypical teratoid/rhabdoid tumors (ATRT) are highly aggressive neoplasms with a unique cytogenic profile. In the literature there is only one case-report of intraspinal ATRT. Here we report another case of a 4-year-old girl with an ATRT C5/6/7.

Methods: A 4-year-old girl was admitted to us with a tetraplegia. Initially there had only been pain in the right arm. MRI of the spinal axis showed an intradural, right-sided tumor C5/6/7. Surgical intervention was undertaken to detach the spinal cord via posterior approach. After right-sided hemilaminectomy of C5–7 we performed an intraspinal and intradural tumor extirpation. Immediately after surgery, strength and mobility started to improve. Only a par- and dysesthesia of the right arm remained. After knowing the histopathology of ATRT we conducted another MRI of the spine. It showed a tumor ventral to the myelon C5/6/7. To try a complete resection we performed a ventral corporectomy C6, extirpation of the tumor, and fusion with a fibula implant.

Results: The tumor could be completely resected microsurgically with the second operation. The postoperative course was uneventful, and a further chemotherapy and radiation were added. Several MRI scans after the second surgery showed complete tumor regression. 7 months after the first diagnosis MRI showed metastases and tumor progress with wide meningeal spread. The spinal ATRT spread into lung, liver, and neuroaxis. 10 months after primary diagnosis the girl died.

Conclusions: ATRT were previously associated with an extremely poor prognosis, but more recent evidence with complete surgical resection and adjuvant chemotherapy shows extended survival in some cases, supporting an aggressive and comprehensive approach to give these patients the best chance for a good outcome. In the current literature a spinal ATRT is described in only one more case report.