원발성 종격동 비정상피종성 생식세포종 3예

Three cases of primary mediastinal Nonseminomatous germ cell tumors

저자들은 젊은 연령의 성인 남자들에서 종격동에 발생한 원발성 종격동 비정상피종성 생식세포종 3예를 수술적 제거 및 화학요법을 통해 치료하던 중 암표식인자의 의미있는 감소와 종양 크기의 감소를 얻었으나 종양 재발에 대한 보조 화학 요법후 합병증이 발생한 경험을 하였기에 이를 보고하는 바이다.

Primary mediastinal nonseminomatous germ cell tumor is extremely rare. Apart from rarity and large size, mediastinal germ cell tumors show striking similarity to testicular tumors in age, incidence, and tumor type. The symptoms associated with these tumors are related mainly to size, invasion of neighboring structures, and distant metastases. Tissue diagnosis is obtained by biopsy of the primary lesion or by biopsy of metastatic sites. Tumors often present with advanced bulky disease, which are unresectable. So these tumors require an aggressive multidisciplinary approach to management. Optimal management includes aggressive surgical debulking and early use of cisplatin-bleomycin-based combination chemotherapy. Serial biomarker measurements permit early recognition of recwrence and improved timing of surgical intervention. The prognosis for mediastinal germ cell tumors is poor, not only because they are far advanced at the time of diagnosis but also because some of the tumors-such as embryonal carcinomas, choriocarcinomas, and endodermal sinus tumors-are very aggressive. In these cases, we present three young male patients with large mass on anterior mediastinum. Tissue diagnosis was obtained by primary lesion biopsy. All patients received surgical debulking and combination chemotherapy and experienced a brief response and eventually had relapses. We report these cases with a review of literatures.