Chordoma in the lateral medullary cistern in a patient with tuberous sclerosis: A case report and review of the literature.

Surgical Neurology International
Kristopher T KimmellEric H Sincoff

Abstract

Chordomas are rare intracranial tumors. There are several reported cases of these tumors arising in patients with tuberous sclerosis (TSC), a neurocutaneous disorder inherited in autosomal dominant fashion that predisposes patients to hamartomatous and neoplastic lesions. A 38-year-old man with the diagnosis of TSC presented with the complaint of dizziness and near syncope. Imaging revealed a mass in the lateral medullary cistern that was found at the time of surgery to be a chordoma. The patient underwent a left far lateral approach for removal of the tumor. Upon opening of the dura, the tumor could be seen under the arachnoid. The tumor was carefully debulked within the limits of safety. The patient did well postoperatively and was referred to the radiation oncology department at our institution for follow-up radiotherapy of the tumor bed. This study presents an unusual presentation and location for a chordoma and contributes to the growing literature associating chordomas with TSC.

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Citations

May 27, 2014·Neuro-Chirurgie·B GeorgeK Beccaria
Sep 7, 2014·Biochimica Et Biophysica Acta·Youssef YakkiouiYasin Temel
May 13, 2017·Journal of Neuropathology and Experimental Neurology·Nathan A DahlJean M Mulcahy Levy
Aug 30, 2018·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Susana Boronat, Ignasi Barber
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Mar 9, 2011·Journal of Neuro-oncology·Roberto Jose Diaz, Michael D Cusimano
Dec 11, 2020·Human Genome Variation·Saba AhmadAkira Yoshii

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Methods Mentioned

BETA
total resection

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