Endocrine Abstracts

Introduction: Sinonasal neuroendocrine carcinoma SNECs are a rare group of neoplasms that account for only 5% of all sinonasal malignancies. SNECs are categorized by their differentiation grade into well-, moderately- and poorly differentiated.

Case report: We describe a classical case of SNEC with secondary orbital involvement in a 34-year-old male patient presented of the occurrence of a right epistaxis. First of all, the patient is smoking. The beginning of the symptomatology goes back to 2 months before with right nasal obstruction of progressive onset, associated with bloody rhinorrhea and right chronic eye watering. The patient didn’t present neurological signs nor cervical lymph nods. Otherwise, ophthalmologic examination found normal visual acuity and ocular mobility on both sides. Sinonasal computed tomography (CT) found a right nasal process enhanced after injection of contrast product 46×30×37 mm invading the orbit. The immunohistochemical study of the tumor biopsy found that the pan cytokeratin and CK7 and the neuroendocrine marker Chromogranin A, synaptophysin and CD56 are positive, with a proliferation index Ki67 of 60%. The multidisciplinary decision taken was treatment by chemotherapy before surgery.

Discussion: Most patients present at advanced stages due to the lack of significant symptoms. Advanced tumors may invade the skull, orbit or brain. Ophthalmic manifestations include exophthalmos, reduced vision and restriction in ocular motility. Ectopic hormone secretion has been described in a handful of cases. Staging is of limited value in predicting prognosis and recent literature clearly highlights the importance of histological diagnosis, particularly differentiation grade, in determining the prognosis and predicting treatment response.

There are no clear treatment and the management include surgery with radiotherapy with/without chemotherapy; chemoradiotherapy; or radiotherapy or chemotherapy alone.