Nomograms for predicting survival and recurrence in patients with adenoid cystic carcinoma. An international collaborative study

Abstract

Background Due to the rarity of adenoid cystic carcinoma (ACC), information on outcome is based upon small retrospective case series. The aim of our study was to create a large multiinstitutional international dataset of patients with ACC in order to design predictive nomograms for outcome. Methods ACC patients managed at 10 international centers were identified. Patient, tumor, and treatment characteristics were recorded and an international collaborative dataset created. Multivariable competing risk models were then built to predict the 10 year recurrence free probability (RFP), distant recurrence free probability (DRFP), overall survival (OS) and cancer specific mortality (CSM). All predictors of interest were added in the starting full models before selection, including age, gender, tumor site, clinical T stage, perineural invasion, margin status, pathologic N-status, and M-status. Stepdown method was used in model selection to choose predictive variables. An external dataset of 99 patients from 2 other institutions was used to validate the nomograms. Findings Of 438 ACC patients, 27.2% (119/438) died from ACC and 38.8% (170/438) died of other causes. Median follow-up was 56 months (range 1–306). The nomogram for OS had 7 variables (age, gender, clinical T stage, tumor site, margin status, pathologic N-status and M-status) with a concordance index (CI) of 0.71. The nomogram for CSM had the same variables, except margin status, with a concordance index (CI) of 0.70. The nomogram for RFP had 7 variables (age, gender, clinical T stage, tumor site, margin status, pathologic N status and perineural invasion) (CI 0.66). The nomogram for DRFP had 6 variables (gender, clinical T stage, tumor site, pathologic N-status, perineural invasion and margin status) (CI 0.64). Concordance index for the external validation set were 0.76, 0.72, 0.67 and 0.70 respectively. Interpretation Using an international collaborative database we have created the first nomograms which estimate outcome in individual patients with ACC. These predictive nomograms will facilitate patient counseling in terms of prognosis and subsequent clinical follow-up. They will also identify high risk patients who may benefit from clinical trials on new targeted therapies for patients with ACC. Funding None.

Parotid adenoid cystic carcinoma: Retrospective single institute analysis

“…Our results support other studies already published in literature [12], based mainly on single-Institution case series with different anatomical sites analyzed [5][6][7][8]. Even multi centric studies reporting wider case-series did not focus on single anatomical site, for instance the REFCOR [4] network provided a large prospective cohort, considering the rarity of ACC, but follow-up was insufficient to assess long-term prognostic factors for overall survival.…”

“…Predictive nomograms, in accordance with Gangly and colleagues [12], could be useful tolls for patient counseling and treatment decision making, which might guide towards more tailored patient care protocol together with the addition of future possible target therapies.…”

“…: age, gender, smoking status and/or alcohol consumption, type of surgery performed with/without adjuvant radiotherapy or chemotherapy, late distant metastasis onset [10,11]. Only one multi center international study [12] has recently published the first nomogram analysis with the aim to predict single ACC patient prognosis using different clinical and pathological parameters never established before.…”

Predictors of Outcome in Adenoid Cystic Carcinoma of Salivary Glands

“…Clinically, the tumor is characterized by a propensity for perineural spread, relentless slow growth, high frequency of local and distal recurrence as well as poor long term survival (1, 5, 9, 11–15, 17, 18). …”

Model to Predict Cause-Specific Mortality in Patients with Head and Neck Adenoid Cystic Carcinoma: A Competing Risk Analysis

“…Finally, authors of an international study of 438 patients constructed nomograms for overall and cause specific survival. 11 In this well-designed study using training and validation cohorts, female sex, younger age, earlier T stage, absence of distant disease and lymph node involvement, negative margins and oral cavity primaries were associated with improved survival.…”