骨盤腔内に多発した膀胱Paragangliomaの1例

Abstract

35歳女.高血圧を主訴とした.23歳時に高血圧の原因精査を行ない, 内分泌検査および画像所見から膀胱および骨盤腔内のparaganglioma疑いを指摘されたが放置していた.今回, 12年前より存在する膀胱paragangliomaの再精査・加療のため当科紹介入院した.経尿道的膀胱腫瘍生検を施行し, 病理組織学的にparagangliomaと診断し, 多発性腫瘍であり両側骨盤腔内リンパ節転移の可能性があったため, 下腹部正中切開, 後腹膜アプローチで膀胱部分切除術および骨盤腔内腫瘍摘除術を施行した.病理所見では, 膀胱筋層内に多発性のある大型細胞が胞巣状ないし充実性の増生を認めた.フローサイトメトリーによるDNA量解析では右骨盤腔内腫瘍のみにaneuploidパターンを認めたが, 他の膀胱部および左骨盤腔内腫瘍はdipoidパターンであった.術前に高値であった血中ドーパミン, 血中ノルアドレナリン, 尿中ノルアドレナリンは術後全て正常となり, 現在腫瘍の再発はない

A 35-year-old woman, who had a past history of hypertension due to paraganglioma of the urinary bladder and in the pelvis, was referred to us 12 years after the initial diagnosis of paraganglioma. Before coming to us and during the follow-up after enucleation of myoma uteri, she was again diagnosed as having a bladder tumor by a gynecologist. Cystoscopy revealed a non-papillary broad-based tumor, measuring 2 cm in diameter, on the trigone of the urinary bladder. Magnetic resonance imaging also showed two solid tumors, each measuring 2 cm in diameter, on the bilateral peri-vesical sides in the pelvis. Endocrinologic examination disclosed increased levels of serum and urinary catecholamines. Histopathologic examination revealed that the bladder tumor specimen obtained by transurethral resection was paraganglioma. She underwent partial cystectomy and resection of these intrapelvic tumors via a retroperitoneal approach. These tumors were histopathologically diagnosed as multiple paragangliomas. She has been followed up for 10 months after operation without any evidence of recurrence. Herein, we report this rare case of vesical paraganglioma and present a brief review of the relevant literature.