Agressive small cell neuroendocrine carcinoma of the neck: A case report

The location in the neck of small cell neuroendocrine carcinoma (SCNEC) is rare and aggressive with a poor prognosis. We herein describe an extremely uncommon example of cervical SCNEC probably arising in the parotid gland, diagnosed at an advanced metastatic stage.

A 76 year old man, non-smoking, non-alcoholic presented a left cervical and rapidly progressive mass. It was suspicious of malignancy and located in the parotid space and II, III, IV and V lymph node groups. In two weeks, the patient had presented a rapid increase in size, with extensive skin permeation. CT scan revealed a large mass infiltrating the left parotid and submandibular glands compressing the jugulo-carotid vascular axis and liver metastases. Diagnosis was made on the morphological pathological examination supplemented by the immunohistochemistry examination, indeed, cells were immunoreactive for chromogranin A as well as synaptophysin. Chemotherapy based on cisplatin and Etoposide VP 16 was started but the evolution was unfavorable: death occurred after 2 cycles.

Neuroendocrine neoplasm arises from neural crest cells. Its location in the head and neck is extremely rare with a tendency for aggressive local invasion and a strong propensity for both regional and distant metastasis. Treatment may include surgical resection, radiotherapy, chemotherapy, or combination of these modalities. The prognosis is poor.

The clinician must discuss the diagnosis of cervical SCNEC when faced with a man in the seventh decade who presents an agressive rapidly expanding cervical mass. The diagnosis confirmation is pathological combining morphological and immunohistochemical evaluations.