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    ABSTRACT: Primary laryngeal small cell neuroendocrine carcinoma (SCNC) is an unusual malignancy accounting for <0.5% of laryngeal carcinomas. To date, approximately 200 cases of primary and 5 of secondary SCNC of the larynx have been reported. This tumor most often presents in the sixth and seventh decades in men who are heavy cigarette smokers. The lesion may be associated with different paraneoplastic syndromes (ectopic adrenocorticotropic hormone syndrome, Schwartz–Bartter syndrome or syndrome of inappropriate secretion of antidiuretic hormone, and Eaton–Lambert myasthenic syndrome) or with ectopic hormone production. The diagnosis is based essentially on the histologic appearance of the tumor, confirmed by immunocytochemical investigations. Concurrent chemoradiotherapy regimens offer potential for long-term survival. This tumor is biologically aggressive, and the extent of the disease is the most significant independent prognostic factor of survival. The survival rate is similar to that with pulmonary SCNC. © 2008 Wiley Periodicals, Inc. Head Neck, 2008
    Head & Neck 04/2008; 30(4):518 - 524. DOI:10.1002/hed.20797 · 2.64 Impact Factor
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    ABSTRACT: Neuroendocrine tumors are rare neoplasms arising from neural and epithelial origin. The case records of 4 patients with the diagnosis of neuroendocrine laryngeal tumor were retrospectively reviewed. In this analysis of our medical records, we describe a series of 4 men with neuroendocrine laryngeal tumors treated in our department since 1994, including the first extremely aggressive and lethal laryngeal paraganglioma reported in the English-language literature. We also discuss the classification, the macro and microscopical characteristics, clinical and pathologic findings, and treatment of these neoplasms. Although neuroendocrine laryngeal tumors account for approximately 1% of all neoplasms in the larynx, its majority represents very aggressive tumors showing a capacity for metastasis and portending poor outcome. © 2012 Wiley Periodicals, Inc. Head Neck, 2012
    Head & Neck 06/2013; 35(6). DOI:10.1002/hed.22955 · 2.64 Impact Factor
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    ABSTRACT: 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.
    Head & Neck 03/2014; 37(5). DOI:10.1002/hed.23666 · 2.64 Impact Factor
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