The importance of multimodality therapy in the treatment of sinonasal neuroendocrine carcinoma.

Sinonasal carcinoma with neuroendocrine differentiation (SCND) is a rare group of tumors known for their aggressive behavior and poor response to treatment. The data in the literature are sparse and cover a wide range of therapeutic approaches over a protracted timeline. Therefore, it is important that institutions report on their experience with these rare neoplasms. Clinical data, such as age at diagnosis, gender, tumor subtype and stage, treatment intention and modality, recurrence, salvage treatment, and survival of patients with a SCND, diagnosed at our department between 1980 and 2010, were retrospectively analyzed. Fifteen patients were available for analysis; eight with sinonasal undifferentiated carcinoma (SNUC), five with sinonasal neuroendocrine carcinoma (SNEC), and two with small cell neuroendocrine carcinoma (SmCC). The median age at the time of diagnosis was 68 years (range 28-87). Treatment consisted of surgery (2), radiotherapy (4), a combination of these modalities (6) and palliation (3). The estimated 5-year overall survival was 60 % for SNEC, 44 % for SNUC and 0 % for SmCC. According to our institutional experience an aggressive multi-modality approach incorporating (neoadjuvant) chemoradiotherapy, radical surgery and elective treatment of the neck is the best treatment strategy for SCND. The high propensity for distant metastasis and poor prognosis of SmCC warrants consideration of the impact of treatment on the remaining quality of life in these patients.