腎血管性高血圧を合併した小児異所性褐色細胞腫の1例

Abstract

重症高血圧が発見された7歳女児で, 尿中ノルアドレナリン排泄及び血漿ノルアドレナリン値が上昇していた.血漿レニン活性は著しく上昇していた.左腎門部塊と左腎動脈狭窄が認められ, この塊はMRIのT2強調画像で極めて明るい病変を示した.DMSA腎シンチでは左腎の摂取率は4.7%と低かった.左腎動脈狭窄を伴う異所性褐色細胞腫と診断し左腎動脈下面及び大動脈側面に接して存在する27×23×13 mmの腫瘍と左腎を摘出した.電顕で腫瘍内に特徴的神経分泌顆粒を認めた.左腎動脈壁の線維症は軽度であった.術後, 血中ノルアドレナリン値がやや高い以外は全て正常化した

A 7-year-old female was discovered to be severely hypertensive. Urinary noradrenaline excretion and plasma noradrenaline level were elevated. Plasma renin activity was markedly elevated. She was found to have a mass in the hilus of the left kidney and left renal artery stenosis. Magnetic resonance imaging (MRI) of the mass revealed an extremely bright lesion on T2 weighted image. DMSA renal scintigraphy revealed a low uptake rate (4.7%) in the left kidney. A diagnosis of extra-adrenal pheochromocytoma associated with left sided renal artery stenosis was made. The mass and left kidney were removed. Electronmicroscopic examination of the mass revealed characteristic neurosecretory granules. There was only slight fibrosis in the wall of the removed left renal artery.