Endocrine Abstracts

We report a case of small cell neuroendocrine carcinoma of laryngeal localization. The involvement of the larynx still exceptional. We present in this paper the radiological, pathological and therapeutic aspects of this tumor that was diagnosed in a 48-year-old man. A 59 years old male patient, chronic smoker, with 10 packs per year; without any particular medical history. he consulted for a rapidly progressive cervical swelling since one year, associated with dysphonia. The whole evolving in a context of apyrexia and alteration of the general state (asthenia, weight loss considered very important not quantified with a preserved appetite). There was no dysphagia, no dyspnea, no carcinoid syndrome or other paraneoplastic syndromes. The patient consulted an ear nose and throat physician, who ordered a cervicothoracic computed tomography showing thickening of the right vocal chord with two nodular lesions measuring 8×10 mm for the largest one, with multiple cervical ADPs visible in the jugulocarotid artery, measuring 23 mm on the right and 15 mm on the left, with necrotic content. Rigid laryngoscopy under sedation revealed a regular submucosal thickening of the right vocal cord extending superiorly. With presence of jugulo-carotid adenopathies. The anatomopathological study of the biopsy showed: an undifferentiated carcinoma, largely necrotic, infiltrating the fibrous tissue with vascular emboli at the level of the labelled specimen. A second specimen of the right vocal cord discreetly reworked by fibrosis without neoplastic localization at this level is associated. This morphological aspect must make us eliminate an aerodigestive primary. With immunohistochemical complement morphological aspect and immunohistochemical profile in favor of an infiltrating small cell neuroendocrine carcinoma (anti AE1/AE3: diffuse positivity/anti chromogranin A: diffuse and intense positivity/anti synaptophysin: diffuse positivity of moderate intensity/anti -KI67: estimated at 60%). Complementary tests showed a very high carcinoembryonic antigen level of 156.4 ng/ml, i.e. 26 times normal. The tumor extension assessement showed secondary iliac osseous lesions. The octreoscan was not performed due to lack of reagent. Given the histological nature of the tumor which contraindicates surgical treatment, the case was discussed with the oncologists with the decision to stop the investigations and to refer the patient for urgent radio-chemotherapy.

Keywords: larynx, small cell neuroendocrine tumor, endoscopy, radiology, pathology, treatment.