*Oncology Coordination Centre, Grenoble University Hospital, Grenoble, France; §Medical Oncology Clinic, Grenoble University Hospital, Grenoble, France; †INSERM Unit 823, Institut Albert Bonniot, Grenoble, France; ‡Pneumology Clinic, Multidisciplinary Thoracic Oncology Group, Grenoble University Hospital, Grenoble, France; ‖Endocrinology and Nutrition, Grenoble University Hospital, Grenoble, France; and ¶Medical Intensive Care Unit, Grenoble University Hospital, Grenoble, France.
J Thorac Oncol. 2014 Apr;9(4):497-505. doi: 10.1097/JTO.0000000000000116.
Paraneoplastic Cushing's syndrome (CushingPS) in small-cell lung cancer is rare but severe.
We studied 383 patients with small-cell lung cancer diagnosed between 1998 and 2012. Among them, 23 patients had CushingPS, 56 had other paraneoplastic syndrome (OtherPS), and 304 had no paraneoplastic syndrome (NoPS).
After comparison of the three groups, we observed that CushingPS patients had more extensive disease: 82.6% versus 67.8% versus 53.3% (p = 0.005), respectively, with more than two metastatic sites: 63.2% versus 15.8% and 24.1% (p ≤ 0.001), a higher World Health Organization performance status (2-4): 73.9% versus 57.1% versus 43.7% (p = 0.006), greater weight loss (≥10%): 47.8% versus 33.9% versus 16.4% (p ≤ 0.001), reduced objective response to first-line treatment: 47.6% versus 74.1% versus 71.1% (p = 0.04), and poorer sensitivity to first-line treatment: 19% versus 38.9% versus 48.6% (p = 0.01). NoPS patients, with World Health Organization performance status of 3-4, had extensive disease at diagnosis, with response, sensitivity to first-line treatment, and survival similar to the CushingPS group. At relapse, the CushingPS group had no objective response to second-line treatment versus 25% versus 42.8% in OtherPS and NoPS groups, respectively (p = 0.005). The median survival of CushingPS patients was 6.6 months versus 9.2 months for OtherPS and 13.1 months for NoPS patients (p ≤ 0.001). CushingPS is a prognostic factor of death (hazard ratio, 2.31; p ≤ 0.001).
CushingPS is the worst form of the paraneoplastic syndromes with particularly extensive tumors. Reduced objective response and sensitivity to first-line treatment and no response to second-line treatment suggest starting palliative care early at first line and exclusively at relapse.
小细胞肺癌伴副肿瘤库欣综合征（CushingPS）较为罕见，但较为严重。
我们研究了 1998 年至 2012 年间诊断的 383 例小细胞肺癌患者。其中，23 例患者患有 CushingPS，56 例患者患有其他副肿瘤综合征（OtherPS），304 例患者无副肿瘤综合征（NoPS）。
三组比较后，我们发现 CushingPS 患者的疾病更为广泛：82.6%、67.8%、53.3%（p=0.005），转移灶超过两个的比例分别为 63.2%、15.8%、24.1%（p≤0.001），患者的世界卫生组织体能状态（2-4 级）分别为 73.9%、57.1%、43.7%（p=0.006），体重减轻≥10%的比例分别为 47.8%、33.9%、16.4%（p≤0.001），一线治疗的客观缓解率分别为 47.6%、74.1%、71.1%（p=0.04），一线治疗的敏感性分别为 19%、38.9%、48.6%（p=0.01）。NoPS 患者的体能状态为 3-4 级，诊断时疾病广泛，与 CushingPS 组的反应、对一线治疗的敏感性和生存情况相似。复发时，CushingPS 组二线治疗无客观缓解，而 OtherPS 和 NoPS 组分别有 25%和 42.8%的患者有客观缓解（p=0.005）。CushingPS 患者的中位生存时间为 6.6 个月，OtherPS 患者为 9.2 个月，NoPS 患者为 13.1 个月（p≤0.001）。CushingPS 是死亡的预后因素（危险比，2.31；p≤0.001）。
CushingPS 是副肿瘤综合征中最严重的一种，其肿瘤更为广泛。一线治疗的客观缓解率和敏感性降低，二线治疗无反应，提示应尽早开始一线姑息治疗，仅在复发时使用。
