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Neurological Surgery No Shinkei Geka Volume 47, Issue 8 （August 2019）

Neurological Surgery 脳神経外科 47巻8号（2019年8月発行）

Japanese English

症例

原発性トルコ鞍内軟骨様脊索腫の1例 A Case of Primary Intrasellar Chondroid Chordoma 榊原陽太郎 ¹ , 田口芳雄 ¹ , 中村歩希 ¹ , 小野寺英孝 ¹ , 和久井大輔 ¹ , 池田哲也 ¹ , 相田芳夫 ² Yohtaro SAKAKIBARA ¹ , Yoshio TAGUCHI ¹ , Homare NAKAMURA ¹ , Hidetaka ONODERA ¹ , Daisuke WAKUI ¹ , Tetsuya IKEDA ¹ , Yoshio AIDA ² ¹聖マリアンナ医科大学横浜市西部病院脳神経外科 ²聖マリアンナ医科大学横浜市西部病院病理診断科 ¹Division of Neurosurgery, St. Marianna University School of Medicine, Yokohama City Seibu Hospital ²Department of Pathology, St. Marianna University School of Medicine, Yokohama City Seibu Hospital キーワード： chordoma , chondroid , intrasellar , pituitary , diplopia Keyword: chordoma , chondroid , intrasellar , pituitary , diplopia pp.901-907

発行日 2019年8月10日 Published Date 2019/8/10

DOI https://doi.org/10.11477/mf.1436204042

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Abstract 文献概要
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参考文献 Reference

Ⅰ．はじめに

　脊索腫は，胎生期の脊索組織の遺残から発生する腫瘍と考えられ^7,11,13)，約35%は頭蓋内，特に斜台先端部に発生する^{1,3,8,9,11,13)}．

　われわれは，過去2回の手術で下垂体腺腫と診断したトルコ鞍内原発軟骨様脊索腫の1例を経験した．初期診断の誤りを再考し，かつトルコ鞍内原発脊索腫について考察を加え報告する．

　OBJECTIVES:Intracranial chordomas are thought to arise from remnants of the notochord and usually occur at the parasellar region. We present a case of a primary intrasellar chondroid chordoma in a patient who was initially diagnosed with a pituitary adenoma.

　CASE:A 77-year-old woman had a history of two surgeries for a tumor in the sella turcica（17 months after the 1^st surgery). On initial MRI, the intrasellar mass showed low signal intensity on T1WI, very high signal intensity on T2WI, and inhomogeneous enhancement. On bone reconstructive CT, the sellar floor was thin, and no abnormalities were observed at the top of the clivus. Transsphenoidal surgery was performed. The pathological diagnosis was pituitary adenoma in both cases. Seventy-two months after the 1^st（31 months after the 2^nd）surgery, she developed right-sided oculomotor and abducent nerve palsies again. Since recurrence occurred during the relatively short period, the surgical specimens obtained from the 1^st and 2^nd surgeries were reexamined. Reexamination of the previously obtained specimen demonstrated areas of chondroid tissue that were embedded in a mucoid stroma and tumor cells that were composed of round or pleomorphic nuclei with vacuolated cytoplasm（physaliphorus cells）that were compatible with chondroid chordoma. The third surgery was performed. Postoperatively, her symptoms improved, and cyber knife therapy was administered for the residual part of the tumor.

　CONCLUSIONS:Although intrasellar chondroid chordomas are extremely rare, they should be considered in the differential diagnosis of tumors located in the sella turcica.