Primary small cell neuroendocrine carcinoma of the larynx: a case report

Introduction Small cell neuroendocrine carcinomas (SCNC) of the larynx are a rare tumor entity with a 5-year over-all survival of only 5 % after standard-therapy with radiochemotherapy.

Methods A systematic review of the literature was performed for “SCNC” and “SCNC in head and neck”. Results: Randomised controlled trials are missing for the evaluation of the standard-therapy. Common therapy approaches are largely based on experiences with small cell lung cancer. 0,5 % of all SCNC occur in the head and neck region. In the last 12 years, we diagnosed 9 patients with SCNC in our department, two of these were located in the larynx. We report the case of a 29-year-old man with initial diagnosis of a SCNC of the larynx. The staging showed a lymph nodes metastasis of the neck; distant metastasis was not detected. A radiochemotherapy with cisplatin/etoposid was initiated with a complete response. A re-staging 6 months later revealed several pulmonary node lesions. 6 cycles of palliative chemotherapy with cyclophosphamid, adriamycin and vincristin were administered resulting in a partial remission. Treatment was modified to nivolumab due to progressive disease. This decision was based on phase II results of small cell lung cancer. The survival time of the described patient is currently 18 months.

Conclusion So far there are no clinical reports concerning the use of nivolumab in third-line-therapy of the SCNC. An NTRK (neurotrophic tyrosine receptor kinase) fusion or the folate receptor expression analysis should be considered in order to evaluate the use of a tropomyosin-receptor-kinase-inhibitor or a radionuklid-therapy.

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Publication History

Article published online:
10 June 2020