Moderately differentiated neuroendocrine carcinoma (atypical carcinoid) of the larynx: A clinically aggressive tumor

Objectives: To evaluate the presentation, treatment, and outcome of a small series of patients with moderately differentiated neuroendocrine carcinoma (MDNC) of the larynx seen at one institution and to provide information on this uncommon laryngeal malignancy for therapeutic decision making. Study Design: Retrospective analysis of medical records. Cases were identified from databases maintained by the medical informatics and pathology departments. Methods: Patient, tumor, treatment, and outcome data were collected from medical records. Main outcome measures were recurrence, overall and disease-specific survival, and cause of death. Results: Eleven patients (6 males; 5 females) seen between 1990 and 2003 had sufficient documentation for further evaluation. Patients presented with dysphagia, throat pain, or hoarseness, and all tumors arose in the supraglottis. Eight of 11 patients gave a history of cigarette use. Of the 10 patients treated with curative intent, 7 who recurred all died with disease. The average disease-free interval was 54 months, with a range of 2 to 101 months. The overall and disease-specific survival was 64 and 54 months, respectively. Nine patients were alive 3 years after diagnosis. No correlations with age, stage at presentation, or initial treatment modality and outcome (recurrence or survival) were found. Conclusions: Laryngeal MDNC is an aggressive malignancy with frequent regional and distant metastases. Although surgical resection is usually recommended, patients did respond to radiation and chemotherapy, suggesting a combined approach may be indicated. Aggressive initial therapy is needed because development of recurrence portends a poor outcome.