MEN1型下垂体腺腫の治療経験—7症例の報告

Surgical Treatments for 7 Pituitary Adenomas in Multiple Endocrine Neoplasia Type 1

I．はじめに

　多発性内分泌腺腫瘍症multilple endocrine neo-plasia（MEN）は，複数の内分泌腺に腫瘍性病変を生じる疾患である．罹患する内分泌腺の組み合わせにより1型と2型に分類される．MEN1型はWermer症候群とも呼ばれ，下垂体前葉，副甲状腺および膵島細胞に過形成ないし腺腫を生じる常染色体優性遺伝疾患である^10,11）．下乗体腺腫を生じると脳神経外科での治療の対象になる．発生頻度は欧米では人口1万から10万に1人とされているが^12），本邦では1995年までに143例の報告があるに過ぎず，きわめて稀な疾患と考えられてきた^14）．しかし近年本邦でも，発生頻度は少なくとも10万人あたり1.8人あると推定され，MEN1型はこれまで考えられてきた程稀ではないと報告されている^4）．本邦の脳神経外科領域では，これまでMEN1型の下垂体腺腫に関してまとまった症例の報告はない．そこで今回われわれは本疾患の理解を深め，治療に関与する脳神経外科の立場を知るために，下前体腺腫摘出術を行ったMEN1型自験例7症例を分析した．

We analyzed 7 pituitary adenomas in patients with multiple endocrine neoplasia type 1 (MEN1). Their incidence was 1.6% among 450 patients with pituitary adenomas which had been treated surgically in our department between 1978 and 1999. The age, gender, symptoms, type of hormone secretion, pathological and operative findings in the MEN1 patients were not apparently different from those in the non-MEN1 patients. Incidence of non-functioning pituitary adenomas, however, was more frequently encountered in our series than that in previous reports among Caucasian people. One patient who had 5 previous opera-tions for the MEN tumors died following postoperative subarachnoid hemorrhage. Four of the seven pa-tients presented initial symptoms related to pituitary adenomas and increased serum Ca level was retros-pectively recognized in three of the four at the time of treatment for the pituitary adenomas.