Small Cell Neuroendocrine of the Head and Neck: A Rare Presentation and Review of the Literature

Abstract

Head and neck tumors account for roughly 3% of malignancies in the United States and about 90% of these tumors are squamous cell cancers. Neuroendocrine neoplasms arise from neural crest cells and are commonly found in the gastrointestinal tract. Neuroendocrine neoplasms arising from the head and neck tend to be rare. In this article, we present a rare case of human papilloma virus–associated poorly differentiated small cell neuroendocrine carcinoma (NEC). Our patient was a 62-year-old African American man who presented with worsening left-sided neck pain and swelling that started 3 months prior to presentation, associated with an unintentional 20-pound weight loss over 6 months, hoarseness in his voice, in addition to dysphagia and odynophagia. Biopsy of left-sided tongue mass revealed poorly differentiated small cell NEC that was positive for HPV (E6/E7) RNA in situ hybridization. Patient was found to have metastatic disease at the time of diagnosis and given the aggressive nature of small cell NECs and the patient’s symptomatic burden, chemotherapy with cisplatin and etoposide was initiated in the hospital. The patient was subsequently discharged from the hospital and is continuing treatment outpatient with cisplatin, etoposide, and atezolizumab.

Giant laryngeal neuroendocrine neoplasm causing airway obstruction: A case report and literature review

“…Additionally, the literature reports the successful use of monoclonal antibodies targeting PD-L1 to treat cases of laryngeal NENs, but this still requires confirmation through large-scale clinical trials. [23]…”

Small cell carcinoma with neuroendocrine differentiation of subglottic larynx- a case report

“…The study concluded that the addition of atezolizumab in the first-line chemotherapy resulted in improved overall and progression-free survival (32). Niforatos et al described the use of atezolizumab with cisplatin and etoposide in a patient with head and neck small cell neuroendocrine cancer with good response (33). Moreover, durvalumab (anti-PD-L1) has been showed to significantly improve overall survival in patients with an extensive stage small cell lung cancer in a recent phase 3 clinical trial (CASPIAN) (34).…”

Neuroendocrine Neoplasms of the Head and Neck: A Case Report on an Oropharyngeal Presentation

“…According to the literature, the improved access to diagnosis by imaging and endoscopy seems to have led to an earlier diagnosis and, therefore, an increase in more localized cases without metastases at the time of detection [ 6 ]. As infrequent entities, references to NEN of the head and neck in the literature include isolated cases or very limited series, most of them referring to NECs [ 4 , 7 ]. While treatment recommendations for this condition are not well established, multidisciplinary interventions, including surgery, chemo, and radiotherapy, are often suggested.…”

“…As infrequent entities, references to NEN of the head and neck in the literature include isolated cases or very limited series, most of them referring to NECs [4,7]. While treatment recommendations for this condition are not well established, multidisciplinary interventions, including surgery, chemo, and radiotherapy, are often suggested.…”