Nonsalivary sinonasal adenocarcinoma

Abstract

Thirteen cases of primary non-salivary gland adenocarcinoma of the nasal cavity and paranasal sinuses were studied at UCLA over 20 years. All pathologic specimens were reviewed and those tumors that were histologically distinct from the more common salivary gland-derived tumors were included in the study. Three classifications were identified: well, moderately, and poorly differentiated adenocarcinoma. A distinct variant of sinonasal adenocarcinoma was the intestinal type. The clinical behavior of the latter resembled the well or moderately differentiated types, with behavior mainly predicted by the extent of the disease. These groups have prognostic significance, with the poorly differentiated group having the most virulent course. Nine of 13 tumors occurred in the ethmoid sinuses and all were aggressive locally. Only one case had distant metastases (nodal neck disease in a terminal case). Of five long-term survivors (median five-year follow-up), all had extensive surgical resections and three had full-course radiotherapy. The single most important factor in the treatment of these lesions is adequacy of surgical margins. Four of six patients with confirmed negative margins were cured despite extensive tumors. Three survivors had the cribriform plate taken and one required a combined intracranial/extracranial approach for tumor resection. There were no survivors in four patients treated with primary irradiation.