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

Introduction: 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. Presentation of case: 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. Clinical discussion: 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. Conclusion: 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.