Endoscopic and Open Surgical Techniques Combined with Radiation and Chemotherapy in the Treatment of Sinonasal Undifferentiated Carcinoma (SNUC)

Sinonasal undifferentiated carcinoma (SNUC) is an uncommon, highly aggressive, and clinicopathologically distinctive carcinoma of uncertain histogenesis first described by Frierson et al in 1986. These tumors are destructive locally, frequently metastasize (often to the brain), and have previously been shown to have a very poor prognosis. Initial symptoms of nasal bleeding and nasal obstruction are often ignored or attributed to more benign etiology, delaying diagnosis. Simple nasal speculum examination is often misleading, but prompt endoscopic examination and biopsy by an experienced clinician confirms the diagnosis. Pathologic specimens are generally recognized as high grade anaplastic malignancy, but arriving at the diagnosis of SNUC is challenging. In this retrospective cohort study, we review how a combination of multidisciplinary evaluation and aggressive surgical intervention combined with chemoradiation has been used to treat, and in some cases cure, this rare and highly morbid disease.

Since 2002 nine patients with SNUC have been evaluated and treated at our institution. In this cohort, all patients underwent a surgical resection by endoscopic, endoscopic combined with open, or open techniques alone. Operatively, all attempts were made to resect all visible tumor through both open visualization and the microscopic view endoscopes allow. All patients also underwent radiation therapy alone or in combination with chemotherapy. The timing and order of treatment varied, depending on the patient's condition at diagnosis, and tumor size and location. Some lesions were initially deemed too extensive for resection or the patient too ill to survive radical surgical procedures. When indicated, such patients were medically optimized and then neoadjuvant radiation and/or chemoradiation was utilized to decrease the tumor bulk to make surgical resection possible.

These patients are medically and surgically very complex, and successful treatment required a multidisciplinary treatment team including Otolaryngology, Neurosurgery, Medical Oncology, Radiation Oncology, and Pathology specialist with extensive head and neck experience. At our institution such a team has been in place for 23 years. Patients are presented at our weekly multidisciplinary tumor conference attended by representatives of all of these specialties and a treatment plan is devised by consensus. We have found that urgent evaluation and treatment is essential as the rapid growth of SNUCs can make surgical resection increasingly difficult. Priority is often given to these patients due to this urgency. Flexibility was employed as combined radiation therapy and chemotherapy was extremely difficult for these patients and complicated by frequent comorbidities. Rest periods were sometime required. In addition, we have found that patients and their families tend to cope with treatment better when counseling and antidepressants were provided, and this was frequently an integral part of the patients' therapy.

We have shown that with a combination of multidisciplinary evaluation, aggressive surgical intervention and combined chemoradiation, some positive results are possible. Three of our patients have shown no evidence of disease for several years and three more are clinically negative for shorter periods. Most patient have experienced some value from our intervention in terms of prolonged quality of life, but this has come at the cost of difficult and aggressive treatment.