Department of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA 02215, USA.
Department of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, Boston, MA 02215, USA.
Oral Oncol. 2020 Jul;106:104690. doi: 10.1016/j.oraloncology.2020.104690. Epub 2020 Apr 11.
Little is known about the long-term impact of local and systemic therapies for recurrent/metastatic (R/M) adenoid cystic carcinoma (ACC), or the clinical significance of molecular alterations.
We identified 72 R/M cases among 123 ACC patients from our institution. We report long-term outcomes, predictors of recurrence and survival, and the impact of sequential cancer-directed therapy among R/M patients. We integrate genomic data for 36 sequenced ACC patients.
Median overall survival (OS) from initial diagnosis was 35.1 ys (95%CI: 25.8-37.3) for R/M ACC patients. 10-y OS among R/M patients was 84.7%, worse for patients with extra-pulmonary metastatic disease (p = 0.02). Only initial disease stage predicted recurrence (OR 1.69, p = 0.03). Longer time to first R/M treatment predicted improved survival (p < 0.01); those treated ≤ 3 years from their R/M diagnosis had poor outcomes (p = 0.01). R/M patients who received systemic therapy vs. active surveillance had similar survival (p = 0.35). Molecular findings predicted outcomes: 10-y OS: 100% MYB, 53.3% PI3K, 32.1% NOTCH1 and others, p = 0.03. PI3K mutations predicted a longer disease-free interval (p = 0.04).
Underlying disease biology remains the strongest predictor of outcomes in R/M ACC. Shorter time to R/M therapy predicts poor outcomes. Molecular alterations are prognostic, and PI3K mutations identify an intermediate-risk ACC subgroup.
对于复发性/转移性（R/M）腺样囊性癌（ACC）的局部和全身治疗的长期影响，或者分子改变的临床意义知之甚少。
我们从我们的机构中确定了 123 例 ACC 患者中的 72 例 R/M 病例。我们报告了 R/M 患者的长期结果、复发和生存的预测因素，以及 R/M 患者接受连续癌症靶向治疗的影响。我们整合了 36 例测序 ACC 患者的基因组数据。
R/M ACC 患者从初始诊断到总体生存（OS）的中位时间为 35.1 年（95%CI：25.8-37.3）。R/M 患者的 10 年 OS 为 84.7%，肺外转移性疾病患者的情况更差（p=0.02）。只有初始疾病分期预测复发（OR 1.69，p=0.03）。首次 R/M 治疗时间越长，生存时间越好（p<0.01）；R/M 诊断后 3 年内接受治疗的患者预后较差（p=0.01）。接受全身治疗与积极监测的 R/M 患者的生存情况相似（p=0.35）。分子发现预测了结果：10 年 OS：100%的 MYB、53.3%的 PI3K、32.1%的 NOTCH1 和其他，p=0.03。PI3K 突变预测无疾病间期较长（p=0.04）。
在 R/M ACC 中，基础疾病生物学仍然是预后的最强预测因素。R/M 治疗的时间越短，预后越差。分子改变具有预后意义，PI3K 突变可识别中危 ACC 亚组。
