日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 32(3): 251-256 (2016)
doi:10.9794/jspccs.32.251

症例報告Case Report

Everolimus投与後に心臓横紋筋腫が急速に退縮した結節性硬化症の1乳児例Rapid Regression of Cardiac Rhabdomyoma after Everolimus Administration in an Infant with Tuberous Sclerosis

1大阪市立総合医療センター小児循環器内科Department of Pediatric Cardiology, Osaka City General Hospital ◇ Osaka, Japan

2大阪市立総合医療センター小児不整脈科Pediatric Electrophysiology, Osaka City General Hospital ◇ Osaka, Japan

3大阪市立総合医療センター小児神経内科Pediatric Neurology, Osaka City General Hospital ◇ Osaka, Japan

受付日:2015年11月29日Received: November 29, 2015
受理日:2016年4月28日Accepted: April 28, 2016
発行日:2016年5月1日Published: May 1, 2016
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mTOR阻害薬のeverolimusは,結節性硬化症に伴う腎血管筋脂肪腫や上衣下巨細胞性星細胞腫に対する有効性が報告されているが,心臓横紋筋腫に対する報告は稀である.症例は生後10か月の男児.胎児期から両側側脳室上衣下結節と多発性心臓腫瘍を指摘され結節性硬化症(TS)と診断された.出生後,小さな心臓腫瘍は自然退縮したが,右室中隔,左室自由壁,左室心尖部の大きな腫瘍は残存した.流入路,流出路狭窄はなかった.生後3か月から難治性痙攣が出現し,頭部MRIで上衣下巨細胞性星細胞腫を認めたため,生後10か月から同腫瘍に対してeverolimusを開始した(3.0 mg/m2/日).その結果,上衣下巨細胞性星細胞腫は縮小し,痙攣も抑制できた.心臓腫瘍は,everolimus投与後1か月で左室自由壁の腫瘍が消失し,投与後7か月で左室心尖部の腫瘍も消失した.残存した右室中隔の腫瘍もeverolimus投与前は最大23.4×16.7 mmあったものが,投与後1か月で16.1×4.3 mmまで急速に縮小した.Everolimusは,TSに合併した心臓横紋筋腫を急速に退縮させる可能性がある.

The mTOR inhibitor, everolimus, has been reported to be effective against renal angiomyolipoma and subependymal giant cell astrocytoma, but its use for cardiac rhabdomyoma has been rarely reported. Here we report the case of a 10-month-old male infant who was diagnosed with tuberous sclerosis (TS) during the fetal stage after being identified with subependymal nodules in both the lateral ventricles and multiple cardiac tumors. After birth, the small cardiac tumors regressed, but the larger tumors persisted on the right ventricular septum and on the free wall and apex of the left ventricle. There was no stenosis of inflow or outflow routes. Intractable convulsions occurred 3 months after birth. Subependymal giant cell astrocytoma was found using cranial magnetic resonance imaging. Therefore, everolimus (3.0 mg/m2/day) was initiated 10 months after birth to treat the tumors. This resulted in regression of the subependymal giant cell astrocytoma and controlled the convulsions. The cardiac tumor on the left ventricular free wall disappeared after 1 month of everolimus administration, and the tumor on the apex of the left ventricle disappeared after 7 months. The tumor persisting on the right ventricular septum was 23.4×16.7 mm before everolimus was administered, but decreased to 16.1×4.3 mm after 1 month of its administration. Everolimus may cause rapid regression of cardiac rhabdomyoma that accompanies TS.

Key words: everolimus; rhabdomyoma; cardiac tumor; tuberous sclerosis

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