Asai Shiko, Katabami Takuyuki, Tsuiki Mika, Tanaka Yasushi, Naruse Mitsuhide
Division of Metabolism and Endocrinology, Department of Internal Medicine, St. Marianna University School of Medicine Yokohama City Seibu Hospital, 1197-1, Yasashicho, Asahi-ku, Yokohama, Kanagawa, 241-0811, Japan.
Division of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center, 1-1, Mukaihatacho, Fukakusa, Fushimi-ku, Kyoto, 612-8555, Japan.
Horm Cancer. 2017 Apr;8(2):108-118. doi: 10.1007/s12672-017-0284-7. Epub 2017 Jan 20.
Evidence has not been established to support that combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) improves survival in patients with malignant pheochromocytoma and paraganglioma (M-PPGL). To investigate the efficacy of CVD for this disease, we retrospectively analyzed data of 23 patients with metastatic and unresectable M-PPGL (mean age, 41.7 ± 15.4 years) who received at least 2 cycles of this regimen. The follow-up period after initiation of CVD ranged from 0.3 to 13.7 years, with a median of 3.3 years. CVD therapy achieved a complete tumor response (CR) in 1 patient (4%), a partial response (PR) in 5 (22%), stable disease (SD) in 5 (22%), and progressive disease (PD) in 13 (52%), respectively. All of the responders (CR and PR) but 6% of the non-responders (SD and PD) showed substantial biochemical improvement. The progression-free survival period in the responders was significantly longer than in the non-responders (p < 0.01). Although the overall survival and survival after the diagnosis of M-PPGL were longer in the responders than the non-responders, the difference was not statistically significant (p = 0.08). The progression-free and overall survival period were significantly longer in the non-progression group (CR, PR, and SD) than in the progression group (PD) (1.7 ± 3.3 vs. 0.3 ± 0.3 years, p < 0.01, and 4.6 ± 3.6 vs. 2.0 ± 3.7 years, p = 0.01, respectively). It is therefore suggested that CVD chemotherapy could be useful in controlling tumor progression and improving survival in patients with metastatic and progressive M-PPGL.
尚无证据支持环磷酰胺、长春新碱和达卡巴嗪（CVD）联合化疗可提高恶性嗜铬细胞瘤和副神经节瘤（M-PPGL）患者的生存率。为研究CVD对该疾病的疗效，我们回顾性分析了23例转移性和不可切除性M-PPGL患者（平均年龄41.7±15.4岁）的数据，这些患者接受了至少2个周期的该治疗方案。开始CVD治疗后的随访期为0.3至13.7年，中位数为3.3年。CVD治疗分别使1例患者（4%）达到完全肿瘤缓解（CR），5例（22%）达到部分缓解（PR），5例（22%）疾病稳定（SD），13例（52%）疾病进展（PD）。除6%的无反应者（SD和PD）外，所有反应者（CR和PR）均显示出显著的生化改善。反应者的无进展生存期明显长于无反应者（p<0.01）。尽管反应者的M-PPGL总体生存期和诊断后的生存期长于无反应者，但差异无统计学意义（p=0.08）。非进展组（CR、PR和SD）的无进展生存期和总体生存期明显长于进展组（PD）（分别为1.7±3.3年对0.3±0.3年，p<0.01；4.6±3.6年对2.0±3.7年，p=0.01）。因此，提示CVD化疗可能有助于控制转移性和进展性M-PPGL患者的肿瘤进展并提高生存率。
