神経節芽細胞腫を伴った副腎複合型褐色細胞腫に原発性上皮小体機能亢進症を合併した1例

Abstract

55歳女.右副腎腫瘍の精査加療を主訴とした.検診超音波検査で右副腎腫瘍が発見され, 内分泌学的検査所見で血漿中のアドレナリン, ノルアドレナリンの軽度上昇と一日尿中アドレナリンおよびVMAの排泄量上昇を認めた.画像診断では腹部CTで右副腎に径4cm大の腫瘤を認め, 右副腎原発褐色細胞腫と診断し, 経腹的右副腎摘除術を施行した.H-E染色にて, 血管結合織による胞巣状構造で多形性に富み多核細胞もみられる褐色細胞腫と, それに混在する形で多様な分化段階を示す神経芽細胞と神経線維を背景とし大型の神経節細胞類似の腫瘍細胞とから成る神経節芽細胞腫が認められ, 神経節芽細胞腫を伴った副腎複合型褐色細胞腫と診断した.術後, 上皮小体機能亢進症の画像的評価を行なったところ, 99mTC-MIBIにて集積亢進, 頸部超音波検査にてhypoechoic lesionを認め, 経過観察していたが, 患者希望により約1年後に上皮小体摘除術を施行した.調べ得た限りで本症例は神経節芽細胞腫を伴った副腎複合型褐色細胞腫の7例目の本邦報告例で, 上皮小体機能亢進症を合併した報告例は過去になかった

We report a case of composite pheochromocytoma-ganglioneuroblastoma in the adrenal gland with primary hyperparathyrodisim. A 55-year-old woman consulted our hospital for an examination of a right adrenal tumor, incidentally found by screening abdominal ultrasound sonography. On the clinical diagnosis of pheochromocytoma in the right adrenal gland from the findings of enhanced abdominal computed tomography, endocrinal examinations and 123I-metaiodobenzyl-guanidine scintigram, right adrenalectomy was performed transperitoneally. Histopathological diagnosis was an adrenal composite pheochromocytoma-ganglioneuroblastoma. This combination of compound adrenal tumor is extremely rare, and to date this case may be the seventh reported in Japan. Moreover, since her serum calcium level and intact parathyroid hormone level were high, so we considered the existence of multiple endocrine neoplasia type 2A. 99mTc-methoxyisobutylisonitrile scintigram and ultrasound of the neck revealed hyperparathyroidism, but medullary thyroid carcinoma was not detected. One year later, she was readmitted for parathyroid tumor excision, and histopathological finding was parathyroid adenoma. We concluded that she had both adrenal composite pheochromocytoma and hyperparathyroidism incidentally.