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PURPOSE: Approximately one half of patients with Lambert-Eaton myasthenic syndrome (LEMS) have small-cell lung carcinomas (SCLC), aggressive tumors with poor prognosis. In view of its profound impact on therapy and survival, we developed and validated a score to identify the presence of SCLC early in the course of LEMS. PATIENTS AND METHODS: We derived a prediction score for SCLC in LEMS in a nationwide cohort of 107 Dutch patients, and validated it in a similar cohort of 112 British patients. A Dutch-English LEMS Tumor Association Prediction (DELTA-P) score was developed based on multivariate logistic regression. RESULTS: Age at onset, smoking behavior, weight loss, Karnofsky performance status, bulbar involvement, male sexual impotence, and the presence of Sry-like high-mobility group box protein 1 serum antibodies were independent predictors for SCLC in LEMS. A DELTA-P score was derived allocating 1 point for the presence of each of the following items at or within 3 months from onset: age at onset ≥ 50 years, smoking at diagnosis, weight loss ≥ 5%, bulbar involvement, erectile dysfunction, and Karnofsky performance status lower than 70. The area under the curve of the receiver operating curve was 94.4% in the derivation cohort and 94.6% in the validation set. A DELTA-P score of 0 or 1 corresponded to a 0% to 2.6% chance of SCLC, whereas scores of 4, 5, and 6 corresponded to chances of SCLC of 93.5%, 96.6%, and 100%, respectively. CONCLUSION: The simple clinical DELTA-P score discriminated patients with LEMS with and without SCLC with high accuracy early in the course of LEMS.

Original publication

DOI

10.1200/JCO.2010.32.0440

Type

Journal article

Journal

J Clin Oncol

Publication Date

01/03/2011

Volume

29

Pages

902 - 908

Keywords

Adult, Age Distribution, Age of Onset, Aged, Cohort Studies, Female, Humans, Incidence, Karnofsky Performance Status, Lambert-Eaton Myasthenic Syndrome, Logistic Models, Lung Neoplasms, Male, Middle Aged, Multivariate Analysis, Netherlands, Predictive Value of Tests, Prospective Studies, Severity of Illness Index, Sex Distribution, Small Cell Lung Carcinoma, Survival Analysis, United Kingdom