腎血管筋脂肪腫に下大静脈内腫瘍血栓を合併した結節性硬化症の1例  [in Japanese] INFERIOR VENA CAVAL TUMOR THROMBUS IN A PATIENT WITH RENAL ANGIOMYOLIPOMA ASSOCIATED WITH TUBEROUS SCLEROSIS  [in Japanese]

症例は34歳・女性,主訴は腹部腫瘤.1987年5月,結節性硬化症に合併した右腎血管筋脂肪腫の破裂の診断で,超選択的腎動脈塞栓術を受けた.1991年5月,腹部腫瘤の増大のため当院を受診し,CT,MRI,超音波検査,血管造影で両腎に多発する血管筋脂肪腫,右腎より右心房直下に達する下大静脈内腫瘍血栓を認め,レノグラムでは右腎機能の著明な低下を認めたため,右根治的腎摘除術および腫瘍血栓除去術を施行した.腫瘍血栓は12cm長で,下大静脈壁との癒着はなく,組織学的には脂肪成分が中心であった.腎血管筋脂肪腫に合併した下大静脈内腫瘍血栓の報告は,自験例が7例日であり,(1)全例が女性であり,(2)1例を除いて右腎より発生しており,(3)腎血管筋脂肪腫の大きさが腫瘍血栓の発生に関与している可能性は低く,(4)腫瘍血栓は下大静脈切開術にて摘出可能であることを指摘した.

A 34-year-old woman with tuberous sclerosis presented with an increase of an abdominal mass and intermittent left flank pain on May 20, 1991. Computed tomography showed multiple bilateral renal masses with fatty density areas and a fatty density thrombus in the inferior vena cava, which extended through the right renal vein of the right kidney on ultrasonography. The inferior vena caval thrombus was also demonstrated by magnetic resonance imaging. Since marked deterioration of the right renal function was found on renography, right radical nephrectomy with thrombectomy was performed on July 2. Microscopically all tumors were identical with angiomyolipoma. She was discharged on Jury 20 and has been followed with good renal function at the outpatient clinic for more than 2 years. Follow up CT revealed no interval changes in the left renal masses.