Case report: a rare Neoplasia of Larynx – highly malignant neuroendocrine carcinoma

Abstract The primary neuroendocrine carcinom is an uncommon head and neck malignancy and appears only in approximately 1% of all malign tumours in the larynx. They are aggressive tumors with a high rate of metastases. The exact classification is still debated. According to the latest classification, NEC in Larynx are divided into 3 subtypes: well differentiated (G1) NEC-L (Synonym: Carcinoid), moderately differentiated (G2) NEC-L (synonym: atypical carcinoid) and poorly differentiated (G3) NEC-L, which additionally divided into small cell (sm) NEC and large cell (lm) NEC. The G1 NEC-L occurs most rarely, followed by G3 type, whereas the G2 NEC-L is the most frequent one. The distinction into these subtypes influences the therapeutic regime, because clinical behavior and response to treatment differ significantly between the subtypes.

Case report We are describing the clinical progression of a68-year-old man complaining of dysphagia and intermittent hoarseness. Laryngoscopy a tumour mass could be seen at the interarytenoid notch,with an enlargement to the postcricoid area on the right side. Staging demonstrated a poorly differentiated large cell neuroendocrine carcinoma (NEC-L G3 lc). He has been treated by radiotherapy and polychemotherapy with Carboplatin/Etoposide (6 courses) . 12 months after the primary therapy the patient is still free of any complaints and a rezidiv.

Conclusion Therapeutic decisions should be made primarily interdisciplinary and having performed a subtype-specification. Patients with a G3 sm- and lc-NEC-L usually develop early distant metastases, but not in our patient. In cases like ours, the treatment is similar to that of neuroendocrine lung carcinomas and consists of a combination of radiotherapy and chemotherapy.

 

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Conflict of Interest

The author declares that there is no conflict of interest.

Publication History

Article published online:
24 May 2022