褐色細胞腫に神経節細胞腫を合併した副腎髄質原発複合型腫瘍の1例

Abstract

63歳女.糖尿病での加療入院中の腹部CTで右副腎に径5cm大の腫瘍を認め, 精査加療目的で紹介入院となった.内分泌学的検査所見および腹部造影CT・MRIと123I-metaiodobenzyl-guanidineシンチグラフィ所見から右副腎原発褐色細胞腫と診断し, 経胸腰的右副腎摘出術を施行した.病理組織学的所見ではH-E染色にて腫瘍細胞は血管結合織による胞巣状構造で胞体を有する好塩基性顆粒を含んだ褐色細胞腫と好酸性の広い胞体を有する神経節細胞への分化を示す大型細胞を含んだ神経節細胞腫から成っていた.両者間の境界は明瞭で, 7対3の比率で併存する形を成しており, 明らかな悪性所見は認められなかった.以上より本症例は褐色細胞腫に神経節細胞腫を合併した副腎髄質原発複合型腫瘍と診断した.術後経過は順調で, 血糖は内服薬なしで正常値となり, 内分泌学的諸検査も正常化した為, 術後3週間で退院となり外来にて経過観察中である

We report a case of a compound adrenal medullary tumor. A 63-year-old woman was referred to our hospital for examination of a right adrenal tumor, incidentally found by abdominal computed tomography (CT). CT and magnetic resonance imaging (MRI) revealed a round heterogeneous tumor, 5 cm in diameter, on the upper pole of the right kidney. A view of the total body scan demonstrated the uptake into the tumor after the injection of 123I-metaiodobenzyl-guanidine. Serum and urinary adrenaline levels were slightly elevated, and urinary excretion of vanillylmandelic acid was markedly elevated. Her blood pressure was normal. From these findings, the tumor was suspected to be a pheochromocytoma of the right adrenal gland and was resected reteroperitoneally. Pathological diagnosis was a compound adrenal medullary tumor, which was composed of pheochromocytoma and ganglioneuroma. This combination of the adrenal medullary tumor is extremely rare, and to date this case may be the sixth case in the Japanese medical literature.