Undifferentiated Carcinoma of the Nose and Paranasal Sinuses

Abstract

Among twenty-six “undifferentiated” tumors of the nasal cavity and paranasal sinuses treated from 1970 to 1990 at the Institute of Otolaryngology of Florence University, 13 were ultimately diagnosed as true undifferentiated sinusonasal carcinoma (SNUC) by conventional light microscopy and use of monoclonal antibodies to epithelial membrane antigen and cytokeratins. SNUC patients, who ranged in age from 20 to 82 years, often had multiple sinonasal symptoms due to very large tumors (nine of 13 tumors were staged as T3–T4) with short average delay of 4 months between onset of symptoms and diagnosis. Both data suggest the high growth capacity and aggressiveness of such a tumor. In our series, follow-up evidenced an overall crude 5-year survival rate of 15.5%. Worse prognostic factors are neck metastases and orbital invasion, according to the behavior of more common carcinomas of the nose and paranasal sinuses. We also found a better prognosis for SNUC primarily arisen in the nasal cavity than in paranasal sinuses (crude 5-year survival rate of 66% vs. 10%, respectively). The histopathological and clinical analysis of our series shows that SNUC is a highly aggressive, uncommon tumor of the nose paranasal sinuses, which should be recognized in advance for a more aggressive treatment by combined multiple therapy.

Combined-modality treatment improved outcome in sinonasal undifferentiated carcinoma: single-institutional experience of 21 patients and review of the literature

“…Gallo et al [10] published in 1993 an immunohistochemical study on 13 patients with SNUC and reported briefly on the outcome of these patients treated at the University of Florence between 1970 and 1993, mainly with radiotherapy. Only one patient was treated surgically and four with non-platinum-based chemotherapy.…”

A Clinicopathological Study of Sinonasal Neuroendocrine Carcinoma and Sinonasal Undifferentiated Carcinoma

“…These tumors were previously diagnosed as “anaplastic, or undifferentiated.” The authors originally reported eight patients (one who had a significant exposure to heavy metals and two who were coal miners). Gallo et al 5 reported on 13 patients with SNUC (taken from a group previously classified as “undifferentiated”), with two having worked in the chemical industry and two as shoemakers.…”

“…However, other studies of larger groups of patients with olfactory neuroblastoma reflect the true bimodal age peak of olfactory neuroblastoma with a median age of 49 years. 6 Gallo et al 5 found that the mean age of patients with SNUC was 56.7 years for men and 68 years for women. In our series, the mean age was slightly lower at 49.3 years (both men and women).…”

“…Occasionally, strong epithelial membrane antigen staining is observed in olfactory neuroblastoma, which may cloud the diagnosis. 7 The S100 staining is usually diffuse for SNEC, but occasionally, an olfactory neuroblastoma–like “sustentacular” pattern of staining has been observed 8 The collective 4,5,9 immunohistochemical pattern of expression reported for SNUC is as follows: lymphocyte common antigen in 0 of 43 cases, cytokeratin in 33 of 43, S100 in 1 of 8, NSE in 20 of 43, epithelial membrane antigen in 37 of 43, chromogranin in 14 of 18, and carcino‐embryonic antigen in 8 of 18. With ultrastructural examination, a few very sparsely distributed neurosecretory granules were found in five of five cases.…”

Establishment and Characterization of Novel Cell Lines from Sinonasal Undifferentiated Carcinoma

“…It was first described by Frierson, et al in 1986 (1), and fewer than 100 cases had been reported by 2005 (18). Despite multimodality therapy, including surgical resection, chemotherapy and radiotherapy, the prognosis for patients with SNUC is poor; the median survival is less than 18 months (2, 3) and the 5-year survival is less than 20% (19). …”