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Background
These are rare tumors accounting for about 10-20% of all sinonasal malignancies. In spite of this rarity, these tumors have been associated with a variety of environmental agents leading to fascinating epidemiological studies. Broadly, these tumors are divided into salivary-type adenocarcinomas and nonsalivary adenocarcinomas. The salivary type resembles salivary gland cancers arising in other salivary glands. The most common type is the adenoid cystic carcinoma and typically occurs in the maxillary antrum. The mucoepidermoid carcinoma is the next most common type.
Nonsalivary adenocarcinomas are divided into low-grade adenocarcinoma and intestinal-type adenocarcinoma. Intestinal type adenocarcinoma is the second most common adenocarcinoma following adenoid cystic carcinomas. It occurs predominately in men (85-95%) and predominates in the ethmoid sinus. These tumors have a strong association with prolonged exposure to fine hardwood dusts and less so with leather dust. Sporadic cases usually occur in women and arise in the maxillary antrum. These tumors are locally aggressive with frequent local recurrences in up to 53% of cases. Metastasis to regional lymph nodes occurs in 8% and distant metastasis occurs in 13% of cases. When death occurs, it usually results from uncontrolled local disease or cranial nerve extension. Under the microscope, there are a variety of histologic patterns (see table below). In general, the papillary-tubular types showed a better prognosis. These tumors may mimic small and large intestinal mucosa and may have histologically benign areas merging with areas of an adenoma and finally malignant glands, histologically indistinguishable from intestinal colonic adenocarcinoma.
Papillary-Tubular (Grades I-III)
Goblet cell type
Signet ring cell type
Mixed or transitional typeThe other nonsalivary adenocarcinoma is the low-grade adenocarcinoma with a wide age range of 9-75 years (median 54 years). Unlike the intestinal type, there is no occupational exposure risk. The tumors arise from various sites (see table below).
Site Percentage Nasal cavity 22% Nasal septum 18% Ethmoid or nasoethmoid 30% Maxillary or nasomaxillary 13% Multiple 18% These tumors have a good prognosis with 30% developing local recurrence. In one study following patients for 6 years, 78% of patients were disease free. Under the microscope, these tumors have a bland cytologic appearance and may be mistaken for a benign papilloma. The growth pattern is one of back to back glands and may contain papillary infoldings.
The pathologist is faced with the difficulty in distinguishing these tumors from metastatic adenocarcinomas. Since sinonasal adenocarcinomas are so rare, metastatic adenocarcinomas from such organs as the thyroid, colon, and ovary must be excluded. Immunoperoxidase studies as well as radiologic imaging such as CT and MRI are helpful.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS INCIDENCE
Malignant neoplasms of the nasal cavity and paranasal sinuses: a series of 256 patients in Mexico City and Monterrey. Is air pollution the missing link?Calderon-Garciduenas L, Delgado R, Calderon-Garciduenas A, Meneses A, Ruiz LM, De La Garza J, Acuna H, Villarreal-Calderon A, Raab-Traub N, Devlin R.
Curriculum in Toxicology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
Otolaryngol Head Neck Surg 2000 Apr;122(4):499-508 Abstract quote Air pollution is a serious health problem in major cities in Mexico. The concentrations of monitored criteria pollutants have been above the US National Ambient Air Quality Standards for the last decade.
To determine whether the number of primary malignant nasal and paranasal neoplasms has increased, we surveyed 256 such cases admitted to a major adult oncology hospital located in metropolitan Mexico City (MMC) for the period from 1976-1997 and to a tertiary hospital in Monterrey, an industrial city, for the period from 1993-1998.
The clinical histories and histopathologic material were reviewed, and a brief clinical summary was written for each case. In the MMC hospital the number of newly diagnosed nasal and paranasal neoplasms per year for the period from 1976-1986 averaged 5.1, whereas for the next 11 years it increased to 12.5. The maximal increase was observed in 1995-1997, with an average of 20.3 new cases per year (P = 0.0006). The predominant neoplasms in these series were non-Hodgkin's lymphoma, squamous cell carcinoma, melanoma, adenocarcinoma, Schneiderian carcinoma, and nasopharyngeal carcinoma.
In the Monterrey hospital a 2-fold increase in the numbers of newly diagnosed nasal and paranasal neoplasms was recorded between 1993 and 1998. The predominant MMC neoplasm in this series, namely nasal T-cell/natural killer cell non-Hodgkin's lymphoma, is potentially Epstein-Barr virus related. Nasal and paranasal malignant neoplasms are generally rare. Environmental causative factors include exposure in industries such as nickel refining, leather, and wood furniture manufacturing. Although epidemiologic studies have not addressed the relationship between outdoor air pollution and sinonasal malignant neoplasms, there is strong evidence for the nasal and paranasal carcinogenic effect of occupational aerosol complex chemical mixtures.
General practitioners and ear, nose, and throat physicians working in highly polluted cities should be aware of the clinical presentations of these patients. Identification of this apparent increase in sinonasal malignant neoplasms in two urban Mexican polluted cities warrants further mechanistic and epidemiologic studies.
DISEASE ASSOCIATIONS CHARACTERIZATION OCCUPATIONAL EXPOSURE
Nasal carcinoma in woodworkers: a review.Wills JH.
J Occup Med 1982 Jul;24(7):526-30 Abstract quote Analysis of data from 12 countries revealed that 61.3% of cases of primary cancer of the upper respiratory tract, and 78.5% of those classified as adenocarcinoma, observed in areas in which furniture making or other manipulation of wood was an important occupation, had occurred in woodworkers. Machine sanding of hardwoods may entail an especially high risk of development of cancer within the sinonasal area.
The data suggest that, although the mean period of latency for development of sinonasal cancers is of the order of 40 to 45 years, the period of exposure to wood dust that may result in the development of mucosal neoplasms may be considerably shorter.
Sinonasal cancer and occupational exposure to formaldehyde and other substances.Luce D, Gerin M, Leclerc A, Morcet JF, Brugere J, Goldberg M.
INSERM Unite 88, Paris, France.
Int J Cancer 1993 Jan 21;53(2):224-31 Abstract quote A case-control study of cancer of the nose and paranasal sinuses was conducted in France to determine whether occupational exposure to formaldehyde was associated with an increased risk of sinonasal cancer.
Exposures to 14 other substances or groups of substances were also studied (wood dust, leather dust, textile dust, flour dust, sugar dust, coal/coke dust, nickel compounds, chromium compounds, chromium VI, welding fumes, soldering fumes, cutting oils, paints and lacquers, glues and adhesives). Cases (n = 207) and controls (n = 409) were interviewed to obtain detailed information on job history and other potential risk factors for sinonasal cancer. In addition, a questionnaire specially designed for this study was used to help assess exposures to formaldehyde and other substances of interest. The questionnaires were translated into history of occupational exposure by an expert in industrial hygiene, without knowledge of case-control status.
Several exposure variables (lifetime average level, duration, cumulative level) were used to describe the risk related to exposure to formaldehyde. Potential confounding factors (occupational and non-occupational) were examined and adjusted for when necessary. No significant association was found between exposure to formaldehyde and squamous-cell carcinomas of the sinonasal cavities. Because of the strong association between exposure to wood dust and nasal adenocarcinoma, it was not possible to assess an independent effect of formaldehyde on this type of cancer. However, among males exposed to medium or high levels of wood dust, the risk of adenocarcinoma associated with formaldehyde was significantly elevated for the highest exposure categories for average level (OR = 5.3, 95% confidence interval = 1.3-22.2), cumulative level (OR = 6.9, 95% CI = 1.7-28.2) and duration of exposure (OR = 6.9, 95% CI = 1.7-27.8).
Although a residual confounding effect of wood dust could not be excluded, this study suggests that exposure to both formaldehyde and wood dust may increase the risk of nasal adenocarcinoma, by comparison with the risk due to wood dust alone. This study also indicated an increased risk among males who had been exposed to glues and adhesives, for all histologic types, which was not explained by a confounding effect of paints and lacquers, wood dust or formaldehyde. No other significant association was observed.
Sinonasal cancer and wood dust exposure: results from a case-control study.Leclerc A, Martinez Cortes M, Gerin M, Luce D, Brugere J.
Unite 88, INSERM, Paris, France.
Am J Epidemiol 1994 Aug 15;140(4):340-9 Abstract quote A case-control study of occupational risk factors for sinonasal cancer was conducted in France in 1986-1988. The study included 207 histologically confirmed cases and 409 controls. Among the male cases were 59 men with squamous cell carcinoma and 82 with adenocarcinoma. The risk of sinonasal cancer in relation to wood dust exposure was studied in these two groups.
The analysis was based on a case-by-case assessment of exposure by an industrial hygienist. Hardwood and softwood were distinguished. An approximate twofold increase in risk for squamous cell carcinomas was observed for cases whose first exposure to either hardwood or softwood occurred before 1945; however, the two types of exposure were highly correlated. An exposure to wood dust--from either hardwood alone or hardwood and other kinds of wood--was found for all but two of the 82 male cases with adenocarcinoma. The effects of different elements of exposure to hardwood (duration, level, period) were studied in detail with a logistic model.
Two components of exposure--duration and average level--contributed independently to the overall very elevated risk. Additional exposure to wood other than hardwood did not increase the risk.
Occupational exposure to dust and sinonasal cancer. An analysis of 386 cases reported to the N.C.C.S.F. Cancer Registry.Van den Oever R.
Landsbond Christelijke Mutualiteiten, Brussels, Belgium.
Acta Otorhinolaryngol Belg 1996;50(1):19-24 Abstract quote An analysis of 386 sinonasal cancer cases reported during the 1978-1994 period to the registry of the Christian Sickness Fund is presented. The relationship between this tumor and previous occupational exposure to carcinogens is investigated by a descriptive case study.
Of 386 cases comprising 294 males and 92 females, 139 were adenocarcinomas which in 88 revealed an occupation as woodworker. In 169 sinonasal cancers the professional history indicates an exposure to dust of different origin, but mainly wood, textile, cereals, cement and leather.
The primary tumor of 87 sinonasal adenocarcinomas in woodworkers was in 77% ethmoidal, 12.2% maxillary, 6.8% nasal and 4-sphenoidal. These findings confirm the results of previous reports on sinonasal cancer from other European countries.
Sinonasal cancer and occupation. Results from the reanalysis of twelve case-control studies.Leclerc A, Luce D, Demers PA, Boffetta P, Kogevinas M, Belli S, Bolm-Audorff U, Brinton LA, Colin D, Comba P, Gerin M, Hardell L, Hayes RB, Magnani C, Merler E, Morcet JF, Preston-Martin S, Vaughan TL, Zheng W.
INSERM, U88, Paris, France
Am J Ind Med 1997 Feb;31(2):153-65 Abstract quote A pooled reanalysis of twelve case-control studies on sinonasal cancer and occupation from seven countries was conducted in order to study associations with occupations other than wood- and leather-related occupations.
The pooled data set included a total of 930 cases (680 men and 250 women) and 3,136 controls (2,349 men and 787 women). All the studies included a detailed occupational history for cases and controls. Each job was coded using the same classifications for occupation and industry. Two approaches were used in the analysis: systematic analysis of occupations; a priori analysis using a preestablished list of occupations and industries. The results confirmed associations observed in several studies not included in this analysis.
For agricultural workers, significant excesses were observed for squamous cell carcinoma among women (OR = 1.69) and men (OR = 3.72 for ten years or more of employment as an orchard worker), and adenocarcinomas among men (OR = 2.98 for ten years or more of employment). Associations with textile occupations were observed for adenocarcinoma among women (OR = 2.60) and squamous cell carcinoma among men (OR = 5.09 for fiber preparers, 3.01 for bleachers). Elevated risks for both histologic types were observed among men employed in food manufacturing (OR = 3.25, adenocarcinoma), or as food preservers (OR = 13.9, squamous cell carcinoma), and among men employed as cooks (OR = 1.99, squamous cell carcinoma).
A positive association with squamous cell carcinoma was observed for male transport equipment operators (OR = 1.21), and also with adenocarcinoma for male motor-vehicle drivers (OR = 2.50). A number of other associations were observed in the systematic analysis.
A second primary intestinal-type adenocarcinoma of the sinonasal tract induced by wood dust.Steinhart H, Bohlender J, Pahl S, Steudel WI, Iro H.
Department of Otorhinolaryngology, University of Saarland, Homburg/Saar, Germany.
Rhinology 2000 Dec;38(4):204-5 Abstract quote Intestinal type adenocarcinoma of the sinonasal tract is associated with exposure to wood dust. We report the case of an adenocarcinoma tumor of the left sinonasal area diagnosed in 1998. Nineteen years earlier (1979), an intestinal type adenocarcinoma of the right nasal cavity was diagnosed. The first tumor was treated in 1979 surgically followed by postoperative radiotherapy.
The second tumor showed the same histological features as an intestinal type adenocarcinoma (papillary type). The patient had a history of a 10 year exposure to wood dust (furniture worker), and the latency time of this patient was 48 years in 1998. We suppose that the tumor of the left side is a second primary intestinal type adenocarcinoma.
This case indicates the usefulness of a lifelong follow up of patients with adenocarcinoma of the nose because of the wide range of latency times of these tumors.
Sinonasal cancer and occupational exposures: a pooled analysis of 12 case-control studies.Luc D, Leclerc A, Begin D, Demers PA, Gerin M, Orlowski E, Kogevinas M, Belli S, Bugel I, Bolm-Audorff U, Brinton LA, Comba P, Hardell L, Hayes RB, Magnani C, Merler E, Preston-Martin S, Vaughan TL, Zheng W, Boffetta P.
Inserm Unite 88, Hopital, National de Saint-Maurice, Saint-Maurice, France.
Cancer Causes Control 2002 Mar;13(2):147-57 Abstract quote OBJECTIVE: In order to examine the associations between sinonasal cancer and occupational exposures other than wood dust and leather dust, the data from 12 case-control studies conducted in seven countries were pooled and reanalyzed.
METHODS: The pooled data set included 195 adenocarcinoma cases (169 men and 26 women), 432 squamous cell carcinomas (330 men and 102 women), and 3136 controls (2349 men and 787 women). Occupational exposures to formaldehyde, silica dust, textile dust, coal dust, flour dust, asbestos, and man-made vitreous fibers were assessed with a job-exposure matrix. Odds ratios (ORs) were adjusted for age, study, wood dust, and leather dust, or other occupational exposures when relevant. 95% confidence intervals (CIs) were estimated by unconditional logistic regression.
RESULTS: A significantly increased risk of adenocarcinoma was associated with exposure to formaldehyde. The ORs for the highest level of exposure were 3.0 (Cl = 1.5-5.7) among men and 6.2 (CI=2.0-19.7) among women. An elevated risk of squamous cell carcinoma was observed among men (OR=2.5, CI=0.6-10.1) and women (OR = 3.5, CI = 1.2-10.5) with a high probability of exposure to formaldehyde. Exposure to textile dust was associated with non-significantly elevated risk of adenocarcinoma, among women only: the OR for the high level of cumulative exposure was 2.5 (CI = 0.7-9.0). High level of asbestos exposure was associated with a significantly increased risk of squamous cell carcinoma among men (OR = 1.6, CI = 1.1-2.3).
CONCLUSIONS: The results of this pooled analysis support the hypothesis that occupational exposure to formaldehyde increases the risk of sinonasal cancer, particularly of adenocarcinoma. They also indicate an elevated risk of adenocarcinoma among women exposed to textile dust, and suggest that exposure to asbestos may increase the risk of squamous cell carcinoma.
PATHOGENESIS CHARACTERIZATION GENERAL
- Genetic analysis of sinonasal adenocarcinoma phenotypes: distinct alterations of histogenetic significance.
Yom SS, Rashid A, Rosenthal DI, Elliott DD, Hanna EY, Weber RS, El-Naggar AK.
1Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Mod Pathol. 2005 Mar;18(3):315-9. Abstract quote
Sinonasal adenocarcinomas, a relatively rare entity, are composed of distinctly different morphologic subtypes with variable biological behavior.
To investigate the genetic events associated with their development and clinicopathologic features, we analyzed the alterations in K-ras, APC, beta-catenin, hMLH1 and hMSH2 and p53 genes expression in a cohort of 15 primary tumors comprising the two main sinonasal adenocarcinoma subtypes (enteric and seromucinous). The patients consisted of 13 men and two women, who ranged in age from 50 to 87 years. Tumors were predominantly located in the ethmoid sinus. Eight tumors were Enteric-type, and seven were seromucinous type. Nine patients were smokers and four were nonsmokers; and no information was available on two patients. Two of the eight enteric-type, had K-ras mutation at codons 12A and 12B, and one showed microsatellite instability at BAT-25. Two patients with enteric-type tumors had a history of wood-dust exposure, and one had a K-ras mutation at 12A codon as well as p53 overexpression. No patients with the seromucinous type had any genetic abnormalities, except for overexpression of p53 in two tumors.
Our results show that (1) a subset of enteric-type sinonasal adenocarcinoma shares certain genetic alterations with colonic adenocarcinomas, (2) the seromucinous-type sinonasal adenocarcinoma lacks alterations and may develop through a different pathway, (3) high p53 expression is associated with aggressive tumor features in both subtypes and (4) the enteric-type runs a more malignant course than the seromucinous counterpart.
Sinonasal adenocarcinoma: evidence for histogenetic divergence of the enteric and nonenteric phenotypes.
Choi HR, Sturgis EM, Rashid A, DeMonte F, Luna MA, Batsakis JG, El-Naggar AK.
Chonnam National University, Kwangju, Korea.
Hum Pathol. 2003 Nov;34(11):1101-7 Abstract quote.
Adenocarcinomas of nonsalivary origin represent approximately 10% to 20% of all sinonasal malignancies and are characterized by varying histopathologic features and uncertain histogenesis.
To better understand the histogenesis and phenotypic heterogeneity of these tumors, we performed immunohistochemical analyses for cytokeratin (CK) 7 and CK20 on 12 primary sinonasal adenocarcinomas (SNACs) representing the histopathologic spectrum of these tumors, adjacent normal mucosa, and 2 metastatic adenocarcinomas from colonic primaries. The demographic and clinicopathologic characteristics of our cohort were similar to those in previously published series. Our results indicate that histologically normal respiratory-type epithelium and submucosal seromucous glands show restricted reactivity to CK7. Epithelial metaplasia of surface epithelium associated with enteric SNACs was accompanied by a conversion from CK7 positivity to CK20 positivity. All primary enteric-type carcinomas and the 2 colonic metastases were reactive to CK20, but all nonenteric-type tumors were negative for CK20 (P=0.003) and positive for CK7. In some of the enteric types, coexpression of CK7 and CK20 was noted.
We conclude that (1) nonenteric-type (seromucinous) adenocarcinoma may originate directly from surface respiratory-type epithelium or from seromucous glands, (2) metaplastic transformation of surface respiratory to enteric-type epithelium precedes the development of enteric adenocarcinoma, and (3) coordinate analyses of CK7 and CK20 reactivity may aid the differential diagnosis of adenocarcinoma in the sinonasal tract.c-erbB2
Prognostic significance of c-erbB-2 oncoprotein expression in intestinal-type adenocarcinoma of the sinonasal tract.Gallo O, Franchi A, Fini-Storchi I, Cilento G, Boddi V, Boccuzzi S, Urso C.
Institute of Otolaryngology-Head & Neck Surgery University of Florence, Italy.
Head Neck 1998 May;20(3):224-31 Abstract quote BACKGROUND: The c-erbB-2 gene codes for a putative transmembrane protein, similar in structure to the epidermal growth factor receptor. Amplification and/or overexpression of the gene has been recently described with a prognostic significance in a variety of human adenocarcinomas.
METHODS: A monoclonal antibody against the c-erbB-2 oncoprotein has been used immunocytochemically in a retrospective study of formalin-fixed, paraffin-embedded samples from 28 consecutive intestinal-type adenocarcinomas (ITACs) of the nose and paranasal sinuses.
RESULTS: Nine out of 28 primary adenocarcinomas (32%) showed positive staining. Clinical follow-up data, available for all patients, suggested in a univariate analysis a correlation between c-erbB-2 expression and poor prognosis, as measured by 5-year disease-free (p = .02) and overall survival curves (p = .07) as well as by recurrence of disease and the appearance of regional and distant metastases (p = .08). In multivariate analysis, c-erbB-2 expression was statistically significant in terms of disease-free survival (p = .046) but not of overall survival (p = .091) in our series.
CONCLUSIONS: These data indicate that c-erbB-2 oncogene activation could be involved in sinonasal tract oncogenesis, with possible prognostic implications.
K-ras
K-ras mutations in sinonasal adenocarcinomas in patients occupationally exposed to wood or leather dust.Saber AT, Nielsen LR, Dictor M, Hagmar L, Mikoczy Z, Wallin H.
National Institute of Occupational Health, Copenhagen, Denmark.
Cancer Lett 1998 Apr 10;126(1):59-65 Abstract quote Of 39 males diagnosed with sinonasal adenocarcinomas over 30 years in the Lund University Hospital catchment area (1.5 million inhabitants), archival tumor tissue was available from 29. Of these, 16 had been exposed to wood dust and three had been exposed to leather dust. The intestinal-type and papillary adenocarcinomas were more common in the exposed patients (P = 0.0002, Fisher's exact test).
The tumors from all but one of the 29 sinonasal adenocarcinomas could be analyzed for point mutations at codons 12, 13 and 61 of the K-ras gene. Four mutations were detected in the 28 tumors. The three mutations in the patients exposed to wood and leather dust were all G:C --> A:T transitions, with two at position 2 of codon 12 and one at position 2 of codon 13.
The high proportion of G:C --> A:T mutations in this rare tumor may reflect a genotoxic agent in wood and leather dust.
LABORATORY/
RADIOLOGIC/
OTHER TESTSCHARACTERIZATION RADIOLOGIC
Optimum imaging for sinonasal malignancy.Lloyd G, Lund VJ, Howard D, Savy L.
Institute of Laryngology and Otology, University College London, UK.
J Laryngol Otol 2000 Jul;114(7):557-62 Abstract quote A combination of computed tomography (CT) and magnetic resonance imaging (MRI) is now established as the optimum assessment of sinonasal malignancy. CT and MRI are of particular value in assessing the skull base, orbit and pteryo-palatine and infratemporal fossae.
Although MRI offers better differentiation of tumour from surrounding tissue and fluid, coronal CT is still required for the demonstration of bone erosion particularly in the region of the cribriform plate. Thus the extent of local tumour spread may be determined with a degree of accuracy in excess of 98 per cent. However, the final determinant of penetration of the dura and orbital periosteum requires per-operative frozen section assessment.
A knowledge of the tissue characteristics and site of origin can be of value in distinguishing some of the commoner sinonasal malignancies such as squamous cell carcinoma, adenocarcinoma, adenoid cystic carcinoma, olfactory neuroblastoma and chondrosarcoma. Imaging, particularly MRI also plays an important role in the post-therapeutic follow-up of patients, indicating areas of residual or recurrent disease, defining suspicious areas for biopsy. Post-operative surveillance is best achieved with three planar T1-weighted MRI, with, and without, gadolinium and axial T2-weighted sequences.
The subtraction of the T1 pre- and post gadolinium T1 sequences can be of particular value in delineating recurrence.
LABORATORY MARKERS
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL
Department of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston, TX 77030, USA.
Nasopharyngeal adenocarcinomas (NPACs) are uncommon neoplasms with a diverse histomorphology and clinical behavior.
The purpose of our study was to better understand the clinicopathologic characteristics of NPACs and to identify the histologic and immunohistochemical features that distinguish the subtypes of papillary NPACs. We conducted a retrospective review of 44 cases of NPACs accessioned between 1985 and 2000. We obtained follow-up information for all patients.
We identified 24 females and 20 males, ranging in age from 9 to 74 years (median, 50 years). There were 28 salivary gland type, 13 conventional low-grade papillary NPACs of surface origin, and 3 metastatic adenocarcinomas, 2 thyroid and 1 lung. We performed immunohistochemical studies in 18 papillary NPACs. Two of the low-grade papillary NPACs simulated thyroid carcinoma; they expressed CK7, CK19, and TTF-1 but were negative for thyroglobulin. Polymorphous low-grade papillary NPACs expressed diffuse reactivity to S-100, whereas low-grade papillary NPACs were negative or focally positive. All patients with low-grade NPACs were alive at 5 to 20 years. In contrast, 18 (64.2%) of the 28 patients with salivary gland-type NPACs had died of the disease or were living with disease at follow-up. Primary NPACs can be classified by their morphology and clinical behavior into 2 groups: surface origin type and salivary gland type.
Papillary NPACs can be identified by their histology and selective immunohistochemical expression. Pathologist should distinguish the different types of NPACs because their treatment and prognosis differ.
Nonsalivary sinonasal adenocarcinoma.Alessi DM, Trapp TK, Fu YS, Calcaterra TC.
Division of Head and Neck Surgery, Jonsson Comprehensive Cancer Center, Los Angeles, CA.
Arch Otolaryngol Head Neck Surg 1988 Sep;114(9):996-9 Abstract quote 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.
Intestinal-type adenocarcinoma of the nasal cavity and paranasal sinuses. A clinicopathologic study of 6 cases.Lopez JI, Nevado M, Eizaguirre B, Perez A.
Service of Pathology, Hospital Civil de Bilbao, Spain.
Tumori 1990 Jun 30;76(3):250-4 Abstract quote The clinical and pathologic features of 6 cases of intestinal-type adenocarcinoma of the sinonasal region are presented.
These cases were collected in a 17 year period (1972-1988) and account for less than 4% of malignancies of this region in our records for this period. All of the patients were men aged 48 to 82 years (mean, 54 years). Previous exposure to wood dust was reported in 1 case. Radiographic studies, especially computerized tomography, were of critical importance to delineate the extent of tumors. Nasal obstruction was the most common complaint.
Duration of symptoms prior to diagnosis is available in 5 cases and ranged from 5 to 36 months (mean 18 months). Surgical treatment was performed in 4 patients (of palliative type in 2) followed by radiotherapy in 3.
Histopathology revealed tubulo-papillary (5 cases) and mucinous (1 case) patterns. Follow-up is available in all patients (range 0 to 108 months), 50% of whom are still alive. In our series, only 1 patient has survived more than 5 years.
Data pooled from the literature reveal that 53% of patients have experienced local recurrences following therapy, and 60% have died of their disease. Of these deaths, 80% occurred within 5 years of diagnosis.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL
Sinonasal tract seromucous adenocarcinomas: A report of 12 cases.Neto Ag A, Pineda-Daboin K, Luna Ma M.
Ann Diagn Pathol. 2003 Jun;7(3):154-9. Abstract quote Sinonasal seromucous adenocarcinomas may originate from the surface epithelium or from the submucosal glands.
We reviewed the clinicopathologic material from 12 patients with sinonasal tract seromucous adenocarcinomas at the University of Texas M. D. Anderson Cancer Center (Houston, TX). There were nine men and three women age 30 to 87 years (mean age, 56.3 years). The clinical presentation included nasal obstruction, nasal mass, and epistaxis. Eight tumors were located in the nasal cavity, three in the ethmoidal sinuses, and one involved the nasal cavity and ethmoid.
Histologically, in nine cases the neoplastic glands were lined by a single cell type, arranged back to back without intervening stroma and often inducing desmoplastic reaction. The remaining three tumors also had a cribriform and papillary pattern. All patients were treated by surgical resection. Three patients had recurrences, which occurred at 36, 36, and 48 months after initial therapy. Their treatment involved surgery and irradiation. Eleven patients are alive and free of disease at 36 to 108 months after diagnosis. One patient died 48 months after diagnosis of another cause.
Sinonasal tract seromucous adenocarcinomas arise purely from submucosal seromucous glands. The diagnosis is facilitated by their anatomic location, the absence of tumor within the mucosal surface epithelium, and the striking similarity to terminal tubules of the seromucous glands.
Histologic classification of sinonasal intestinal-type adenocarcinoma.
Franquemont DW, Fechner RE, Mills SE.
Department of Pathology, University of Virginia Health Sciences Center, Charlottesville 22908.
Am J Surg Pathol 1991 Apr;15(4):368-75 Abstract quote Kleinsasser and Schroeder recently described a histologic classification system for woodworker-associated, intestinal-type adenocarcinomas of the sinonasal region.
To determine if their approach is easily applied and prognostically meaningful for both woodworker-associated and sporadic intestinal-type adenocarcinomas in the sinonasal region, we analyzed 15 such cases. The 12 men and three women ranged in age from 37 to 75 years. Only four were woodworkers. All tumors arose in the nasal cavity or paranasal sinuses. The three authors independently classified the tumors with unanimous agreement in 11 (73%) of 15 cases. Disagreements were resolved by group review and consensus.
Ten tumors were papillary tubular cylinder cell type; these were subdivided into grades I (four cases) and II (six cases) on the basis of cytologic atypia. Three tumors were alveolar goblet cell type; one tumor was signet-ring type; and one had a mixed pattern. Median survivals were papillary tubular I, 9 years; papillary tubular II, 3 years; and alveolar goblet cell, 7 years.
It is concluded that this classification system is easy to apply, reproducible, and appears to identify a group of sinonasal intestinal-type adenocarcinomas (papillary tubular I) with a prolonged survival.
VARIANTS CLEAR CELL CARCINOMA
Primary description of a new entity, renal cell-like carcinoma of the nasal cavity: van Meegeren in the house of Vermeer.Zur KB, Brandwein M, Wang B, Som P, Gordon R, Urken ML.
Box 1189, Mount Sinai School of Medicine, 1 Gustave Levy Pl, New York, NY 10021, USA.
Arch Otolaryngol Head Neck Surg 2002 Apr;128(4):441-7 Abstract quote BACKGROUND: Few sinonasal malignancies can manifest, histologically, as clear cell neoplasia. The most likely such tumor to be encountered is metastatic renal cell carcinoma. Primary sinonasal tumors that can appear as clear cell malignancies include squamous cell carcinoma and mucoepidermoid carcinoma. Primary salivary clear cell carcinoma occurs almost exclusively in the oral cavity and has not been described in the nasal cavity.
OBJECTIVE: To report a unique sinonasal clear cell malignancy that mimicked metastatic renal carcinoma.
STUDY DESIGN: Case report.
OUTCOME MEASUREMENTS: Radiography, histology, histochemistry, immunohistochemistry, and electron microscopy.
RESULTS: Histologically, the tumor was identical to renal cell carcinoma. No evidence of renal malignancy was found by abdominal computed tomographic scan or gadolinium-enhanced magnetic resonance imaging. Histochemistry confirmed the presence of tumor glycogen but no mucin. Immunohistochemistry confirmed strong expression of low- and high-molecular-weight keratin and S100, and no vimentin expression. Electron microscopy showed tumor myofibroblastic differentiation and cytoplasmic glycogen, neutral lipid vacuoles, and cholesterol.
CONCLUSIONS: There was no clinical evidence of renal cell carcinoma. The immunohistochemical and ultrastructural findings were inconsistent with the diagnosis of renal cell carcinoma and showed features also inconsistent with the diagnosis of primary salivary clear cell carcinoma. We therefore conclude that this tumor represents a new and distinct entity, notable in its presentation as a "counterfeit renal cell carcinoma."
Low grade primary clear cell carcinoma of the sinonasal tract.Moh'd Hadi U, Kahwaji GJ, Mufarrij AA, Tawil A, Noureddine B.
Department of Otolaryngology-HNS, American University of Beirut Medical Center, New York, USA.
Rhinology 2002 Mar;40(1):44-7 Abstract quote Clear cell carcinoma of the salivary glands are rare tumors. Metastatic clear cell carcinoma from a primary in the adrenal glands to the head and neck area have been described in the literature. However, primary clear cell carcinoma of the paranasal sinuses have not been yet alluded to in the literature except in the paper of Newman (1993).
We are presenting our experience and long term follow-up in the diagnosis and management of such a lesion in the nose and paranasal sinuses.
LOW GRADE ADENOCARCINOMA
- Sinonasal tubulopapillary low-grade adenocarcinoma: a specific diagnosis or just another seromucous adenocarcinoma?
Luna MA.
From the Department of Pathology, University of Texas MD Anderson Cancer Center, Houston, Texas.
Adv Anat Pathol. 2005 May;12(3):109-15. Abstract quote
Histopathologically, sinonasal adenocarcinomas fall into four categories: the intestinal type, the conventional salivary gland type (eg, adenoid cystic carcinoma, acinic cell carcinoma), the seromucous type, and the low-grade not otherwise specified type.
Recently, a new type of sinonasal adenocarcinoma has been described, called tubulopapillary low-grade adenocarcinoma. In this commentary, the histologic features of this type of tumor are compared with those of the other types of sinonasal adenocarcinoma.
The clinicopathologic characteristics and probable origin of this type of adenocarcinoma are also discussed.
Low-grade adenocarcinoma of the nasal cavity and paranasal sinuses.Heffner DK, Hyams VJ, Hauck KW, Lingeman C.
Cancer 1982 Jul 15;50(2):312-22 Abstract quote The pathologic features and the behavior of 50 cases of adenocarcinoma (excluding adenoid cystic carcinoma and mucoepidermoid carcinoma) of the sinonasal tract are presented.
The cases were divided on histologic grounds into 23 low-grade and 27 high-grade neoplasms. The low grade lesions had a well developed glandular pattern throughout, very uniform nuclei, and minimal mitotic activity. The high grade tumors had a less uniform glandular pattern, commonly with solid or sheet-like areas, manifested nuclear pleomorphism, and generally had a higher mitotic rate. The low-grade group had a prognosis markedly better than the high-grade group.
Since the literature tends to consider all sinonasal adenocarcinomas as relentlessly progressive neoplasms with poor prognosis, it is important to recognize this category of low grade neoplasm in order that treatment and prognostication can be better related to their behavior.
SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE Immunophenotypic Differences Between Intestinal-type and Low-grade Papillary Sinonasal Adenocarcinomas: An Immunohistochemical Study of 22 Cases Utilizing CDX2 and MUC2.
Cathro HP, Mills SE.
Department of Pathology, University of Virginia, Charlottesville, VA.
Am J Surg Pathol. 2004 Aug;28(8):1026-32. Abstract quote
Nonsalivary sinonasal adenocarcinomas can be divided into low-grade and high-grade tumors. The former are often papillary and the latter are usually of intestinal type, morphologically similar to metastatic colonic carcinoma.
Antibodies to CDX2, a transcription factor gene highly specific for intestinal adenocarcinomas, MUC2, a mucin gene expressed in adenocarcinomas from various sites, and cytokeratins (CK) 7 and 20 were used to examine the two groups of tumors. Formalin-fixed, paraffin-embedded tissue from 22 sinonasal adenocarcinomas was reclassified into 9 high-grade intestinal-type, 3 high-grade nonintestinal, and 10 low-grade, predominantly papillary adenocarcinomas. Immunohistochemical staining was graded on a 0 to 4+ scale with 5% or greater tumor cell staining considered positive. Of the high-grade intestinal group, 78% demonstrated 4+ CDX2 positivity, with 44% MUC2 positive. Although 89% of this group was CK7 positive, the percent of staining was variable. A majority (67%) of the intestinal cases was 4+ CK20 positive. Almost every nonintestinal adenocarcinoma (90%) (low- and high-grade) was CK7 positive (7 of 9, 4+), without expression of any of the three colonic adenocarcinoma markers.
The three high-grade nonintestinal tumors had the expression profile of the low-grade papillary group with the exception of focal MUC2 positivity in 1 case. Intestinal-type adenocarcinomas have an expression profile distinct from nonintestinal sinonasal adenocarcinomas. The former are similar, but not identical, to colonic adenocarcinomas.
Immunohistochemical staining for CDX2, MUC2, and differential cytokeratins does not differentiate metastatic colorectal from primary sinonasal intestinal-type adenocarcinoma.
Intestinal-type adenocarcinoma of the sinonasal tract: a clinicopathologic study of 18 cases.Urso C, Ninu MB, Franchi A, Paglierani M, Bondi R.
Istitut di Anatomia e Istologia Patologica, Universita di Firenze, Italy.
Tumori 1993 Jun 30;79(3):205-10 Abstract quote BACKGROUND: Intestinal-type adenocarcinoma (ITAC) of the nose and paranasal sinuses is a relatively rare tumor. It commonly affects subjects exposed to wood or leather dust.
METHODS: The authors present the clinicopathologic findings of 18 cases of sinonasal ITACs and review the proposed histologic classifications.
RESULTS: All patients, except one, were males; mean age was 60 years (range, 41-79); in 9 cases an occupational exposure to wood or leather dust was found. Common presenting symptoms were epistaxis, nasal obstruction and rhinorrhea. Histologically, tumors were divided into four groups: well-differentiated (G1) ITACs = 3 cases; moderately differentiated (G2) ITACs = 8 cases; poorly differentiated (G3) ITACs = 2 cases; mucinous (M) ITACs = 5 cases. Immunocytochemically, 16/17 cases were positive for carcinoembryonal antigen, 1/17 for somatostatin, and 0/16 cases for gastrin.
CONCLUSIONS: Sinonasal ITACs are aggressive tumors, often diagnosed in a relatively advanced stage. Owing the close similarity of the microscopic aspects, a histologic classification of ITACs analogous to that of colonic adenocarcinomas is proposed.
Sinonasal intestinal-type adenocarcinoma: immunohistochemical profile and comparison with colonic adenocarcinoma.McKinney CD, Mills SE, Franquemont DW.
Department of Pathology, University of Virginia Health Sciences Center, Charlottesville, USA.
Mod Pathol 1995 May;8(4):421-6 Abstract quote Sinonasal intestinal-type adenocarcinomas (ITAC), as their name implies, bear a striking resemblance to primary intestinal neoplasia. The value and limitations of immunohistochemistry in making this distinction have not been previously defined.
We determined the immunohistochemical staining profile of 12 sinonasal ITAC and compared their staining with that of 12 histologically similar colonic adenocarcinomas.
All ITAC stained for cytokeratin and epithelial membrane antigen. Additional positive reactions were as follows: B72.3, 11 of 12; Ber EP4, 11 of 12; Leu M1, 8 of 12; HMFG-2, 12 of 12; and BRST-1, weak staining in seven of 12 cases. All 12 ITAC were negative for vimentin, synaptophysin, and actin. Colonic carcinomas stained similarly for these markers. Three additional antigens differed in their expression in ITAC versus colonic tumors. Carcinoembryonic antigen was strongly present in only two of 12 ITAC, with focal positivity in six of 12 and no staining in four of 12 cases.
In contrast, all 12 colonic adenocarcinomas were strongly positive for carcinoembryonic antigen. Chromogranin-positive cells were present and often numerous in nine of 12 ITAC, in contrast to only rare positive cells in three of 12 colonic tumors. Neuron-specific enolase was present in five of 12 ITAC but was absent from all colonic tumors studied. ITAC are less often and less strongly carcinoembryonic-antigen positive and more prone to exhibit divergent neuroendocrine differentiation.
These features may be of some value in distinguishing ITAC and colonic metastases. Neuroendocrine differentiation in ITAC was associated with higher mortality. Of the five patients with ITAC having 1+ to 2+ chromogranin positivity, only one was free of disease.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES METASTATIC RENAL CELL CARCINOMA
An unusual presentation of a metastatic hypernephroma to the frontonasal region.Sgouras ND, Gamatsi IE, Porfyris EA, Lekka JA, Harkiolakis GC, Nikolopoulou SM, Valvis PJ.
Department of Plastic and Reconstructive Surgery, Metaxa's Memorial Cancer Institute, Piraeus, Greece.
Ann Plast Surg 1995 Jun;34(6):653-6 Abstract quote We report a rare case involving an 85-year-old man who presented with a large metastatic hypernephroma to the frontal sinuses, the base of the nose, and the ethmoid sinuses, disfiguring the patient's face. Frequent but intermittent and mild epistaxis was one of the main symptoms. He had no history of renal malignancy, and even at the time of our examination (18 months after the appearance of the facial tumor) he did not have any symptoms of the primary renal carcinoma (not even hematuria).
This metastasis may have occurred through the vertebral plexus of veins that communicate with the great venous plexus of the head and the plexus of the paranasal sinuses. If a metastatic hypernephroma to the sinonasal tract is the only clinical metastasis, as in our patient, a radical excision of the solitary metastasis, together with a nephrectomy, is recommended.
Physicians dealing with head and neck lesions should always suspect a metastatic tumor and especially, a hypernephroma.
RESPIRATORY EPITHELIAL ADENOMATOID HAMARTOMA
*Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA daggerDepartment of Anatomic Pathology, Cleveland Clinic Foundation, Cleveland, Ohio.
Respiratory epithelial adenomatoid hamartoma (REAH) is an unusual benign sinonasal glandular proliferation. REAH is not considered a neoplasm, although, no molecular evidence exists to support or refute this possibility.
Microdissection of 10 cases of REAH, 9 cases of sinonasal adenocarcinoma (SNAC) and 10 cases of chronic sinusitis was performed. DNA was extracted and polymerase chain reaction performed using fluorescently labeled primers flanking known tumor suppressor genes on chromosomes 9p (CDKN2/p16), 11p (H-ras), 17p (p53), and 18q (DCC/DPC4). Polymerase chain reaction products were analyzed semiquantitatively by capillary electrophoresis. Allele ratios were calculated using the peak height from the shorter allele divided by the peak height from the longer allele. The loss of heterozygosity (LOH) ratio was calculated as the allele ratio from tumor tissue divided by the allele ratio from normal tissue. The fractional allelic loss (FAL) was calculated as the percentage of loci that harbored LOH divided by the number of loci that were informative.
REAH demonstrated an intermediate FAL of 31% compared with SNAC (64%) and chronic sinusitis (2%). REAH and SNAC had the highest LOH for multiple loci located on 9p (p16) and 18q (DCC/DPC4). The molecular profile of REAH shows a mean FAL of 31%, which would be considered unusually high for a non-neoplastic entity.
Appreciable allelic loss within REAH suggests the possibility that REAH may be a benign neoplasm rather than a hamartoma.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSTIC FACTORS SURVIVAL
Cancer of the nasal cavity and paranasal sinuses: a series of 115 patients.Svane-Knudsen V, Jorgensen KE, Hansen O, Lindgren A, Marker P.
Department of Otorhinolaryngology, University Hospital, Odense, Denmark.
Rhinology 1998 Mar;36(1):12-4 Abstract quote A total of 115 patients with sinonasal cancer was assessed during the period 1978-1995. Ninety-one patients received treatment with curative intent. A combination of irradiation and operation was used.
The 5-year crude survival for patients, who were treated with curative intent, was 41%; the disease-specific survival throughout the period was 48%. Primary irradiation followed by maxillectomy was widely used in the first half of the period. Treatment in the last part was changed to primary lateral rhinotomy with post-operative irradiation whenever possible.
Twelve maxillectomies were performed during the first half of the period, and during the last part, only two. Disease-specific survival was equal in the first and the second halves of the period.
VASCULAR ENDOTHELIAL GROWTH FACTOR
- Prognostic significance of microvessel density and vascular endothelial growth factor expression in sinonasal carcinomas.
Valente G, Mamo C, Bena A, Prudente E, Cavaliere C, Kerim S, Nicotra G, Comino A, Palestro G, Isidoro C, Beatrice F.
Pathology Section, Department of Medical Sciences, Amedeo Avogadro University Medical School, Novara, Italy.
Hum Pathol. 2006 Apr;37(4):391-400. Epub 2006 Feb 8. Abstract quote
The prognostic significance of microvessel density and proliferative activity of the neoplastic cells, evaluated respectively by CD31 and Ki-67 positivity, and immunohistochemical expression of vascular endothelial growth factor (VEGF) was retrospectively investigated in 105 cases of sinonasal carcinoma (80 surgical specimens and 25 biopsies).
The most represented histologic types were intestinal-type adenocarcinoma found in 36 patients (34.3%), squamous cell carcinoma (SCC) in 34 (32.4%), mucinous adenocarcinoma (mainly made up of signet-ring cell patterns) in 15 (14.3%), and adenoid cystic carcinoma in 7 (6.7%). Microvessel density values (in vessels per square millimeter), VEGF, and Ki-67 were not dependent on histologic type but were rather correlated to the histologic grading in SCC. Clinical data were available for 92 (87.6%) of 105 patients, with minimum follow-up of 48 months. Most of the patients (81.5%) were at an advanced stage (T3-T4) at diagnosis. The values of all markers were correlated to tumor stage (P = .03).
Multivariate analysis showed that both microvessel density and proliferative activity of the neoplastic cells were independent prognostic parameters (mortality hazard ratio, 1.33 and 1.60, respectively). Although VEGF expression was not correlated to prognosis on the whole series (P = .06), it was a powerful prognostic marker when the analysis was restricted to the group of SCCs (hazard ratio, 3.02; 90% confidence interval, 1.58-5.80).
These results show that tumor neoangiogenesis, expressed by microvessel density, together with proliferative activity, is a pathologic marker with a strong prognostic impact in sinonasal carcinomas. Therefore, it may be a useful tool in this field so as to carry out therapeutic protocol planning, which may be further enhanced by the adoption of the more recent antiangiogenic molecules.TREATMENT SURGERY
Craniofacial resection for tumors of the nasal cavity and paranasal sinuses--a 17-year experience.Lund VJ, Howard DJ, Wei WI, Cheesman AD.
Institute of Laryngology and Otology, London, United Kingdom.
Head Neck 1998 Mar;20(2):97-105 Abstract quote BACKGROUND: The rarity of sinonasal tumors has precluded long-term follow-up of large series of craniofacial resections until now.
METHODS: A series of 209 patients suffering from a wide range of histologies who had undergone craniofacial resection for sinonasal neoplasia with up to 17 years' follow-up were analyzed.
RESULTS: An overall actuarial survival of 51% at 5 years and 41% at 10 years was found for the cohort as a whole. For malignant tumors, the 5-year actuarial survival was 44%, falling to 32% at 10 years. For benign pathology, the actuarial survival was 75% at both 5 and 10 years. Statistical analysis identified three factors which significantly affect outcome and survival: malignant histology, brain involvement, and orbital involvement. Few complications are associated with the surgery, with the mean post-operative stay being 16 days.
CONCLUSIONS: The improved survival and minimal morbidity and mortality associated with craniofacial resection make it the optimum approach to sinonasal tumors.
RADIATION
Postoperative radiotherapy for adenocarcinoma of the ethmoid sinuses: treatment results for 47 patients.Claus F, Boterberg T, Ost P, Huys J, Vermeersch H, Braems S, Bonte K, Moerman M, Verhoye C, De Neve W.
Division of Radiotherapy P7, Ghent University Hospital, De Pintelaan 185, 9000 Ghent, Belgium.
Int J Radiat Oncol Biol Phys 2002 Nov 15;54(4):1089-94 Abstract quote PURPOSE: Ethmoid sinus cancer is a rare malignancy. Treatment results are mostly reported together with other sinonasal tumors, grouping a wide range of different histologies and treatment approaches. This study reports on the treatment outcome of 47 patients diagnosed with adenocarcinoma of the ethmoid sinuses and treated with surgery and high-dose postoperative radiation therapy.
METHODS AND MATERIALS: Between September 1985 and October 2001, 51 patients with adenocarcinoma of the ethmoid sinuses were referred to the Ghent University Hospital. Four patients were treated with low-dose palliative radiation because of very extended inoperable disease or distant metastasis at the time of diagnosis. They were not included in this analysis. The other 47 patients, all staged as N0M0, were treated with surgery and postoperative high-dose radiation therapy. The median follow-up was 32 months. The T-stages were T1 for 2, T2 for 17, T3 for 11, and T4 for 17 patients. All 47 patients were staged as N0M0.
RESULTS: The 3-year, 5-year, and 7-year overall survival are respectively 71%, 60%, and 38%. The 3-year and 5-year disease-free survival are respectively 62% and 36%. The 3-year and 5-year disease-free survival for T1-T2 stages are respectively 87% and 55%, for T3 stages 57% and 28%, and for T4 stages 41% and 25%. The locoregional tumor control was 70% and 59% at respectively 3 and 5 years. Patients presenting with intracranial tumor invasion at the time of diagnosis relapsed within 7 months after the end of radiotherapy. Radiation-induced severe dry eye syndrome and optic neuropathy was observed in respectively 7 and 2 of the 47 cases.
CONCLUSION: Postoperative radiotherapy for adenocarcinoma of the ethmoid sinuses is associated with good local control rates. Crucial for a favorable prognosis is the absence of intracranial invasion. The rarity of these tumors makes it difficult to evaluate new therapeutic advances.
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