Neuroendocrine carcinoma (NEC) can be an aggressive disease with locoregional and distant metastasis. We present this article (1) to highlight the typical presentation of NEC in head and neck primary sites such as the parotid gland, paranasal sinuses, and supraglottis and (2) to discuss the prognosis of these tumors based on their histologic subtype and stage. We base our comments on the findings of our retrospective review of the cases of 16 adults-10 men and 6 women, aged 43 to 88 years (mean: 65.8)-who had been diagnosed with pathologically confirmed NEC of the head and neck. Analysis of subtypes revealed that 11 of these patients (68.8%) had presented with poorly differentiated NEC, 4 (25.0%) with moderately differentiated NEC, and 1 (6.3%) with well-differentiated NEC. The most common primary sites were the salivary glands (n = 5; 31.3%), paranasal sinuses (n = 4, 25.0%), and larynx (n = 4). There was no statistically significant difference in survival at 24 months between the patients with moderately differentiated NEC and those with poorly differentiated NEC (37.5 vs. 35.4%; p = 0.86); at the end of the study period, the patient with well-differentiated NEC was still living, 129 months after diagnosis. Taken together, patients with stage I, II, and III disease had a combined survival of 77.8% at 12 months, which was significantly higher (p = 0.023) than the 57.1% survival at 12 months for patients with stage IV disease.
IntroductionNeuroendocrine carcinoma (NEC) is a composite tumor of the nervous and endocrine systems that has a capacity to release neuropeptides into the systemic circulation. On electron microscopy, the tumor cells are noted to contain membrane-bound, electron-dense neurosecretory granules. On immunohistochemical staining, NEC may be positive for chromogranin, synaptophysin, cytokeratin AE1/AE3, neuron-specific enolase, and others.
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