集学的治療が奏功した非セミノーマ性腺外胚細胞腫瘍の1例

Abstract

23歳男.性腺外胚細胞腫を疑い精査加療目的で入院した.後腹膜原発性腺外胚細胞性腫瘍と診断し, 国際胚細胞腫瘍予後分類(IGCCC)でpoor prognosis groupと分類された.胚転移巣が急速に増大したためBED療法を開始し肝転移巣は消失し, 肺, リンパ節転移は60%以上の縮小を認めたが腫瘍マーカーの再上昇を認めたため, 末梢血幹細胞移植(PBSCT)併用の超大量化学療法(HDC)を施行した.肺, 副腎の腫瘍の縮小率は約80%を示し腫瘍マーカーは完全に陰性化し肺転移巣はすべて1cm未満に縮小した.その後, 右精巣の無痛性腫大が出現し, 右高位精巣摘除術を施行した.腫瘍細胞が存在せず原因は明らかでなく, 特発性と考えた.左副腎摘出, 後腹膜リンパ節郭清術を施行し, 悪性所見を認めず追加化学療法は施行せず退院となった.18ヵ月経過後も再発転移を認めていない

A 23-year-old man presented with lumbago as a chief complaint. Computed tomographic (CT) scan revealed multiple lung tumors, multiple liver tumors, bulky retroperitoneal tumors with marked elevation of serum lactic dehydrogenase (LDH), alpha-fetoprotein, and beta subunit of human chorionic gonadotropin (HCG-beta). The patient was referred to our hospital for treatment. Scrotal ultrasonography and physical examination revealed bilateral normal testes. Because of bulky retroperitoneal masses with elevated specific tumor markers as well as bilateral normal testes, our diagnosis led to extra-gonadal germ cell tumor. Because the pulmonary lesion had increased rapidly, chemotherapy was performed without the tumor biopsy. After multiple chemotherapy regimens including BEP (bleomycin, etoposide, cisplatin), high-dose chemotherapy, and TIN (paclitaxel, ifosfamide, nedaplatin), all tumor marker levels fell into within the normal range. The tumor size was decreased remarkably on CT. Then, retroperitoneal lymphadenectomy were performed to confirm whether they still contained viable tumor cells. They contained only necrotic tissues without viable cancer cells by pathological examination. Consequently, the patient has been free of recurrence for 18 months after intensive treatment.