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Clinical management of localized undifferentiated sinonasal carcinoma

our experience and review of the literature

Perri, Francescoa; Della Vittoria Scarpati, Giuseppinae; Ionna, Francob; Longo, Francescob; Montano, Massimoa; Muto, Paoloc; Ravo, Vincenzoc; Giuliano, Mariod,f; Caponigro, Francescoa

doi: 10.1097/CAD.0000000000000707

Undifferentiated sinonasal carcinoma (SNUC) is defined as a small round blue cell tumor that is immunohistochemically distinct from other sinonasal malignancies, such as lymphoma, mucosal melanoma, nasopharyngeal carcinoma, neuroendocrine carcinoma, and olfactory neuroblastoma. SNUCs are very aggressive malignancies, provoking quick destruction of the splanchnocranium structures. Being a very rare neoplasm, there are no prospective clinical trials assessing their treatment strategies, so lots of data are derived by small retrospective trials. Tri-modality treatments (namely those treatments which use together surgery, radiation therapy and chemotherapy) are now considered the best of care for this category of poor prognosis tumors, and whenever possible they should be employed. Despite the tri-modality treatments and the multidisciplinary management, SNUCs are characterized by poor prognosis with a median overall survival reaching 14 months. Ameliorating radiotherapy techniques and performing therapies adapted to the genetics of the disease could represent a promising strategy of therapy in the near future. In this report, we have presented our experience, describing the treatment and the prognosis of four patients seen at our Institution. Moreover, we have performed a review of the literature analyzing the now available therapy options and the possible future strategies.

Departments of aHead and Neck and Sarcoma Oncology

bOtolaryngology and Thyroid Surgery

cRadiation Therapy, IRCCS G. Pascale

dDepartment of Clinical Medicine and Surgery, University Federico II, Naples

eDepartment of Medical Oncology Unit, POS SS Annunziata, Taranto, Italy

fDepartment of Medical and experimental Oncology, Lester and Sue Smith Breast Center, Baylor College of Medicine, Houston, Texas, USA

Correspondence to Francesco Perri, MD, Department of Head and Neck and Sarcoma Oncology, IRCCS G. Pascale 80131, Naples, Italy Tel: +39 081 590 3362; fax: +39 081 590 3824; e-mails:,

Received July 27, 2018

Accepted October 5, 2018

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