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Neurological Surgery No Shinkei Geka Volume 22, Issue 4 （April 1994）

Neurological Surgery 脳神経外科 22巻4号（1994年4月発行）

Japanese English

症例

Turner症候群に合併したPituitary Hyperplasiaの1例 Turner's Syndrome with Pituitary Hyperplasia:A case report 木戸悟郎 ¹ , 宮城敦 ¹ , 渋谷肇 ¹ , 宮上光祐 ¹ , 坪川孝志 ¹ , 澤田達男 ² Goro KIDO ¹ , Atsushi MIYAGI ¹ , Tadashi SHIBUYA ¹ , Mitsusuke MIYAGAMI ¹ , Takashi TSUBOKAWA ¹ , Tatsuo SAWADA ² ¹日本大学脳神経外科 ²日本大学第2病理学 ¹Department of Neurological Surgery, Nihon University School of Medicine ²Second Department of Pathology, Nihon University School of Medicine キーワード： Turner's syndrome , Brain tumor , Meningionma , Pituitary hyperplasia , Dissection of the aorta Keyword: Turner's syndrome , Brain tumor , Meningionma , Pituitary hyperplasia , Dissection of the aorta pp.333-338

発行日 1994年4月10日 Published Date 1994/4/10

DOI https://doi.org/10.11477/mf.1436900817

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Abstract 文献概要
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I．はじめに

　Turner症候群は，①女性型外性器，②原発性無月経，③低身長，④性染色体の異常，を特徴とする代表的な染色体異常疾患で，性染色体の構成は正常X染色体がひとつのみか，あるいはもう1本，XまたはY染色体構造異常を伴うとされている^7,8）．全身的には，大動脈縮窄症に代表される心大血管系の異常の合併頻度が高いとされているが，中枢神経系疾患の合併に関する報告は少ない^{11,18-20,23,30）}．

　今回，われわれは，長期にわたりestrogen製剤による補充療法が施行され，頭蓋内病変としてpituitary hyperplasiaとmeningiomaを合併し，解離性大動脈瘤破裂によって死亡したTurner症候群の1例を経験した．

Turner's syndrome, a sex-chromosome abnormality, is often accompanied by cardiovascular disorders, such as coarctation of the aorta. We encountered a case of Turner's syndrome with meningioma and pituitary hyperplasia which resulted in death from dissection of the aorta.

The patient was 36-year-old female who was dia-gnosed as having Turner's syndrome of mosaic-type at the age of 15. She had accepted sex-hormone replace-ment with estrogen and progesterone over 20 years.

She lost consciousness and was transferred to our in-stitute on June 20th, 1992. She was in shock but her condition began to improve after intensive treatment. CT scan revealed a calcified mass lesion at the left frontal convexity and a markedly enhanced round mass lesion at the suprasellar region. Angiography showed tumor stain of the suprasellar region fed by the pos-terior ethmoidal artery. These tumors were diagnosed as multiple meningiomas.

She developed dyspnea on June 24th and chest X-ray showed right pleural fluid collection and cardiomegaly. This condition was diagnosed as congestive heart failure. Her condition was getting worse and she suffered abrupt cardiac arrest on June 28th. Autopsy revealed dissection of the aorta as the cause of death. The tumor of the convexity was meningioma, and the suprasellar lesion was diagnosed as pituitary hyper-plasia.

It is well known that frequent excess dose injection of estrogen can induce pituitary adenoma or hyperpla-sia in rats. In this case, the presence of pituitary hyper-plasia was thought to be the result of long-term injec-tion of estrogen. The pathogenesis of this case may be the natural course of Turner's syndrome combined with cardiovascular and endocrinal abnormalities and neurogenic neoplasms.