摘要
肺外小細胞癌是種少見的惡性腫瘤，占所有小細胞癌的2.5-4%。常見好發部位爲頭頸部，腸胃道，泌尿生殖器官與女性生殖系統。臨床表現與肺小細胞癌類似，相當惡性且預後不佳。肺外小細胞癌的致癌機轉與其生物分子變化不明。臨床上對於其診斷，分期與治療是一難題。疾病的分期是最重要的存活因子。治療計畫也需考慮腫瘤原發部位。因其少見與治療經驗不足，目前尚未發展治療準則。對於局部腫瘤通常採合併治療，偶見長期存活的患者。但如何併用與治療順序尚待研究。對於廣泛性與復發轉移的患者則使用化學治療。雖其反應率高，但效果持續短暫。更多的基礎與臨床研究將有助於改善其治療結果。
關鍵字
無資料並列摘要
Extrapulmonary small cell carcinoma (EPSCC) is a relatively rare malignancy accounting for only 2.5~4% of all small cell carcinomas. The head and neck, gastrointestinal tract, genitourinary and gynecologic organs are most common tumor sites. EPSCC behaves aggressively like the small cell lung cancer (SCLC) and its prognosis is dismal. Its tumorigenesis and molecular alternations remain poorly understood. The clinical diagnosis, staging and treatment of EPSCC are challenging. The extent of disease is important for prognosis whereas the tumor location affects the choice of treatment modality. Currently there is no standard guideline as yet. Combined modality (surgery, radiation or chemotherapy) treatment has often been applied to limited-stage EPSCCs in which long-term survivors have ever been reported. However, the sequence of these combinations is not well-defined. Palliative chemotherapy is used for extensive and recurrent/metastatic EPSCCs with high response rate but short progression-free interval. More research is warranted for improving the survival.