Clinical Recommendations on the Treatment of Neuroendocrine Carcinoma of the Larynx: A Meta-analysis of 436 Reported Cases

Background: Current recommendations on the treatment of neuroendocrine carcinoma of the larynx (NCL) are based on anecdotal evidence. With this meta-analysis, our purpose was to provide clinicians with more substantiated guidelines in order to improve the treatment outcome of the patients affected with NCL.

Methods: A structured literature search for all research concerning NCL was performed against the MEDLINE and EMBASE databases. Available data was normalized, pooled, and statistically analyzed.

Results: Four hundred thirty-six cases of NCL were extracted from 182 studies, of which 23 were typical carcinoid, 163 were atypical carcinoid, 183 were small-cell neuroendocrine carcinoma, 29 were large-cell neuroendocrine carcinoma, and 38 were unspecified carcinoid tumors. The 5-year disease-specific survival (DSS) was 100% for typical carcinoid, 53% for atypical carcinoid, 19% for small-cell neuroendocrine carcinoma, and 15% for large-cell neuroendocrine carcinoma (p < .001). Patients with an atypical carcinoid treated with surgery had better DSS than those treated with radiotherapy (60% vs 54%; p = .035). Postoperative radiotherapy did not result in better DSS in atypical carcinoid. Patients with an atypical carcinoid, not undergoing surgical treatment of the neck, developed isolated regional recurrence in 30% of cases (p = .001). Radiochemotherapy yielded the best DSS for small-cell neuroendocrine carcinoma compared to other modalities (31% vs 13%; p = .001).

Conclusion: Typical carcinoid can be treated by local excision alone. Atypical carcinoids do not seem to respond well to radiotherapy and are best managed through radical surgical excision in combination with elective neck dissection. Patients with small-cell neuroendocrine carcinoma or large-cell neuroendocrine carcinoma seem to benefit most from chemoradiotherapy.