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BACKGROUND Whole-body hybrid positron emission tomography (PET) imaging is increasingly used for sinonasal tumors. However, only empirical data exist on the additional, clinically relevant information derived from these techniques. METHODS This study included 96 regionalized magnetic resonance imaging (MRI) of the sinonasal tract/neck and separate hybrid FDG-PET/CT or FDG-PET/MRI in 74 patients. Additional radiological information (ARI) obtained from each hybrid examination was analyzed and its clinically relevance was determined. Clinically relevant information (CRI) was categorized with regard to primary tumor site, regional lymph node metastases, distant metastases, second primary tumors, and non-neoplastic findings. RESULTS A total of 45/96 (46.9%) hybrid PET examinations revealed ARI. CRI was found in 32/96 (33.3%) examinations and concerned the primary tumor site (6.1%), regional lymph node metastases (4.1%), distant metastases (14.3%), second primary tumors (7.3%), and non-neoplastic findings (5.1%). CONCLUSIONS Hybrid PET imaging yields additional radiological information translating into clinically relevant information in a substantial proportion of patients with sinonasal tumors.

Objectives Chondro‐osseous respiratory epithelial adenomatoid hamartomas (COREAH) are extremely rare benign lesions in the sinonasal cavity. We aim to (a) report two cases of COREAH and (b) perform a literature review with a summary on previously published cases of COREAH till August 2019. Methods A literature review identified 16 cases, but only 12 cases from publications from 2005 to 2019 were included in analysis. In addition, we report two cases of COREAH from our tertiary academic medical center. Results COREAH is found in patients from 3 to 83 years old. It originates from various sites in the nasal cavity and sinuses but the most common location is the lateral nasal wall. Calcification on imaging is a common finding. Conclusion COREAH is an uncommon entity that is important to recognize and distinguish from other sinister nasal masses. It is a slow growing benign lesion which lacks aggressive features on imaging. Future studies are needed to evaluate the possibility of an underlying genetic predisposition. Level of Evidence 4., Chondro‐osseous respiratory epithelial adenomatoid hamartomas (COREAH) are benign lesions in the sinonasal cavity that are exceedingly rare and literature on COREAH is limited. We aim to (1) report two cases of COREAH and (2) perform a literature review with a summary on previously published cases of COREAH till August 2019.

AIMS Low-grade papillary Schneiderian carcinoma (LGPSC) is a rare and newly described entity of the sinonasal tract. The aim of this study was to evaluate the clinicopathological and molecular characteristics in order to identify typical features for differential diagnosis. METHODS AND RESULTS Of the 3000 cases of sinonasal tumour studied during a period of 6 years, five cases were reviewed and diagnosed as LGPSC. All five patients were female (mean age, 47.8 years; range, 18-64 years) and had undergone multiple surgeries (3-10 surgeries). Both the sinonasal tract and the middle ear were involved in four patients. Nodal metastasis occurred in two patients, and one patient developed a distant metastasis to the left lung. Histologically, tumours had branched and crowded papillae with pushing boundaries. Tumour epithelia were multilayered and arranged in an orderly pattern without cilia. No malignant cytological features were observed in any of the cases. Immunohistochemical findings revealed a scattered distribution of Ki67-positive cells and positive staining for epithelial membrane antigen, mainly in the outermost-layer cells. Human papillomavirus (HPV) DNA was found in two patients and genotyped as HPV type 16. Sanger sequencing did not reveal any epidermal growth factor receptor or Kirsten rat sarcoma viral oncogene homologue gene mutation in the five cases. CONCLUSIONS We report on five new cases of LGPSC, and confirm LGPSC as a new sinonasal carcinoma that behaves aggressively with metastatic potential. The combination of clinical behaviour and typical histological features can distinguish LGPSC from sinonasal papilloma and other carcinomas.

Human papillomaviruses (HPVs) have been associated with a subset of head and neck squamous cell carcinomas (HNSCCs). The aim of this study was to determine the prevalence of HPV DNA in archived formalin-fixed paraffin-embedded tissue from patients with histologically confirmed HNSCCs in a South African cohort. A nested PCR was used for the detection of HPV DNA targeting the L1 gene. Positive samples were confirmed using an in-house hemi-nested PCR targeting the E6 gene and genotyped by sequence determination of amplicons. HPV DNA was detected in 57/780 (7.3%) samples, with the highest prevalence being in the sinonasal tract (16.0%) and oropharynx (10.8%). HPV16 was the most frequently detected type, being found in 26/57 (45.6%) positive samples. The prevalence of HPV DNA in HNSCCs found in this study was lower than that found in developed countries.

OBJECTIVES Extranodal natural killer/T-cell lymphoma (ENKTL) is a highly aggressive tumor of the sinonasal tract associated with poor overall survival (OS). This study expands upon epidemiologic, prognostic, and treatment factors for OS and disease-specific survival (DSS), incorporating newly accessible chemotherapy data. METHODS Retrospective population-based cohort study performed on cases of sinonasal ENKTL identified through the Surveillance, Epidemiology, and End Results database. Univariate Kaplan-Meier analysis and subsequent multivariate Cox-regression analysis were performed to evaluate prognostic and treatment variables for OS and DSS. RESULTS Four hundred and sixty cases of sinonasal ENKTL were identified. Five-year OS and DSS were 46% and 56%, respectively. On multivariate analysis, higher Ann Arbor stage was associated with worse OS (P

OBJECTIVES To further characterize the epidemiology, prognostic disease-specific factors, and treatment outcomes-including newly available chemotherapy data-for patients with sinonasal diffuse large B-cell lymphoma (DLBCL). METHODS The Surveillance, Epidemiology, and End Results registry was queried from 1973 to 2015 for patients with DLBCL of the nasal cavity and paranasal sinuses. Demographic and disease parameters were collected. Prognostic disease-specific survival and overall survival factors were evaluated with univariate Kaplan-Meier analysis. Significant variables were analyzed with multivariate Cox regression analysis. RESULTS A total of 1,273 cases of DLBCL of the sinonasal tract were identified. Significant differences in age of diagnosis between men (65.3) and women (71.1) existed (P

Solitary fibrous tumor (SFT) is a fibroblastic tumor of the pleura, and subcutaneous and deep soft tissues of the extremities, abdomen, pelvis, and retroperitoneum. In the head and neck region, the sinonasal tract and soft tissues of the orbit are involved most frequently. However, the lacrimal sac is an extremely unusual location for this tumor, with occasional case reports in literature. Being superficially located and palpable, lacrimal sac masses are easily amenable to FNAC. Despite cytological features of SFT being well-documented, they are often underdiagnosed on FNAC. Immunocytochemistry for STAT6 is a sensitive and specific marker that can aid in accurate diagnosis, as is required for planning appropriate management. We describe the case of a 45-year-old lady who presented with a mass at the medial canthus of the right eye, and was diagnosed as SFT on FNAC, based on cytomorphological features and immunopositivity for STAT6. The diagnosis was subsequently confirmed on histological examination.

Background Sinonasal sarcomas are rare and heterogeneous in nature. Continuously collecting data influencing the prognosis is fundamental for optimizing therapeutic assessment of this highly destructive neoplasm. Methods We conducted a single-institution retrospective cohort study considering 27 patients with sinonasal sarcoma. Results The overall survival (OS) rates for 1 year and 5 years were calculated as 74% and 36%, respectively. Extent of the primary tumor (P = .010), nodal spread (P = .009), and age (P = .004) significantly reduced the OS. Disease-free survival (DFS) was significantly reduced by age (P = .003), extent of the primary (P = .006), nodal (P = .004), and hematogenous (P = .048) spread. Multimodal therapy including surgery improved the OS and DFS rates (P Conclusion Prognosis is poor due to late disease recognition. However, multimodal therapeutic regimens, including surgery, may improve the outcome.

BACKGROUND The 5-year overall survival rate for patients with sinonasal cancers has remained around 50% for the last 3 decades. Prior studies on head and neck cancers have suggested that 1 reason for poor survival is the frequent development of second primary malignancies (SPMs). The purpose of this study is to assess overall and site-specific risks of SPM following treatment of sinonasal malignancy. METHODS A retrospective, population-based cohort study was performed on 2614 patients in the Surveillance, Epidemiology, and End Results (SEER) database who were diagnosed with primary sinonasal malignancy between 1973 and 2014. Standardized incidence ratios (SIRs) and absolute excess risks (AERs) were calculated to assess risk of SPM relative to incidence in the general population. RESULTS A total of 422 (16.1%) patients with primary sinonasal malignancies developed a total of 480 SPMs. This cohort had a significantly higher frequency of SPMs than expected in the general population (SIR 1.32; 95% confidence interval [CI], 1.20 to 1.44; AER 53.41). Site-specific analyses of SIRs suggested highest risk of malignancy in the sinonasal tract (SIR 75.64; 95% CI, 53.53 to 103.83; AER 17.22), followed by bone, eye and orbit, oral cavity and pharynx, and lung and mediastinum. CONCLUSION Patients with history of sinonasal cancer are at significantly increased risk of developing an SPM. Careful monitoring for development of additional tumors may be warranted.

Background Squamous cell carcinoma (SCC) is the most common malignancy in the sinonasal tract. We present the first population-based analysis that examines geographic differences in demographic and clinical characteristics, socioeconomic factors, treatment modality, and disease-specific survival (DSS) of this entity. Methods All cases of sinonasal squamous cell carcinoma (SNSCC) were queried using the U.S. Surveillance, Epidemiology, and End Results (SEER) registry from 1973 to 2013. Patients were stratified by geographic location and characteristics such as demographics (age, gender, race, metropolitan/nonmetropolitan status, and income), stage at diagnosis, and treatment modality. Survival data were generated using Kaplan-Meier regression analysis. Results In total, 6094 patients were identified; 15.3% were from the East, 16.3% from the Midwest, 19.3% from the South, and 49.1% from the West. Patients from the South were younger (p < 0.001). The South had the highest proportion of patients who were black (p < 0.001), lived in nonmetropolitan areas (p < 0.001), and presented with localized disease (p < 0.001). Southern patients also had the lowest median income (p < 0.05), and were least likely to be treated with both surgery and radiotherapy (p < 0.001). The South exhibited the lowest 20-year DSS compared to all other regions (p < 0.001). Conclusion SNSCC patients from the South had the poorest long-term DSS, despite being most likely to present with localized disease. The South had the highest proportion of patients who were black, resided in rural or urban towns, had the lowest median income, and did not receive standard combination therapy, compared to the East, Midwest, and West.

Aims Human papillomavirus (HPV)-related carcinoma with adenoid cystic-like features is a newly described entity of the sinonasal tract. In this study, we evaluated histomorphology, immunophenotype and molecular testing to identify potentially helpful features in distinguishing it from classic adenoid cystic carcinoma (AdCC). Methods and results We retrospectively collected five HPV-related carcinomas with adenoid cystic-like features and 14 AdCCs of the sinonasal tract. All histological slides were retrieved for morphological evaluation. As comparing with AdCC, HPV-related carcinomas with adenoid cystic-like features were associated with squamous dysplasia of surface epithelium (80% versus 0%, P < 0.01) and the presence of a solid growth pattern (100% versus 29%, P = 0.01), but less densely hyalinized tumour stroma (20% versus 86%, P = 0.02). Squamous differentiation in the invasive tumour was seen in three HPV-related carcinomas with adenoid cystic-like features, two of them showing abrupt keratinization and one with scattered non-keratinizing squamous nests. Diffuse p16 staining in ≥75% of tumour cells was noted in all HPV-related carcinomas with adenoid cystic-like features but in only one AdCC (100% versus 7%, P < 0.01). High-risk HPV testing gave positive results in all HPV-related carcinomas with adenoid cystic-like features (four associated with type 33 and one associated with type 16) but not in AdCCs. MYB rearrangement was tested in four HPV-related carcinomas with adenoid cystic-like features, and all were negative. Conclusions This study has further clarified the histological spectrum of this tumour type, and reports the first HPV type 16-related case. Diffuse p16 staining followed by HPV molecular testing is useful in distinguishing HPV-related carcinomas with adenoid cystic features from classic AdCCs.

Aims Human papillomavirus (HPV) is known as causative for squamous cell carcinoma (SCC) of the oropharynx, but is also found not infrequently in carcinomas of the sinonasal tract. Recently, a subset of these carcinomas was recognized to harbour HPV33 and have a significant morphological overlap with adenoid cystic carcinoma (ACC), a rare and aggressive carcinoma originating in the minor salivary glands. Termed ‘HPV-related carcinoma with ACC-like features’, only nine cases have been reported. To clarify the occurrence of these tumours we screened a large material for the presence of HPV-related ACC-like carcinoma. The identified tumours were characterized immunohistochemically and with fluorescence in-situ hybridization, and clinicopathological information for all cases is presented. Methods and results Forty-seven candidate cases were screened for presence of HPV. Six cases were identified and genotyped as HPV types 33, 35, and 56. All six cases had areas of dysplastic mucosal lining and showed remarkable heterogeneous morphologies. MYB, MYBL1, and NFIB genes were intact and, interestingly, staining for MYB protein was largely negative in contrast to what was found in ACC. One patient experienced a local recurrence 11 years after initial treatment and the remaining five patients were alive without evidence of disease. Conclusion We report six new cases of HPV-related ACC-like carcinoma and found that, although in a small material, the prognosis for these patients seems more favourable than for ACC. For the distinction between ACC and HPV-related ACC-like carcinoma, p16, MYB immunohistochemistry or investigation of MYB, MYBL1 and NFIB gene status are valuable.

Inverted papillomas are tumors of the sinonasal tract with a propensity to recur. Raman spectroscopy can potentially identify inverted papillomas from other tissue based on biochemical signatures. A pilot study comparing Raman spectroscopy to histopathology for 3 types of sinonasal tissue was performed. Spectral data of biopsies from patients with normal sinonasal mucosa, chronic rhinosinusitis, and inverted papillomas are compared to histopathology using principal component analysis and linear discriminant analysis after data preprocessing. A total of 18 normal, 15 chronic rhinosinusitis, and 18 inverted papilloma specimens were evaluated. The model distinguished normal sinonasal mucosa, chronic rhinosinusitis, and inverted papilloma tissue with an overall accuracy of 90.2% (95% confidence interval, 0.86-0.94). In conclusion, Raman spectroscopy can distinguish inverted papilloma, normal sinonasal mucosa, and chronically rhinosinusitis tissue with acceptable accuracy.

Human papillomavirus-related carcinoma with adenoid cystic-like features is a newly described histologic variant of sinonasal tract carcinoma. The implications of this sinonasal malignancy is still being evaluated. There are a limited number of cases reported in the literature, and thus we seek to further characterize this patient population and review the histologic features of this malignancy with the following two cases. The behavior of this entity is as yet uncertain. Laryngoscope, 128:1515-1517, 2018.

Oncocytic sinonasal papillomas (OSPs) are benign tumours of the sinonasal tract, a subset of which are associated with synchronous or metachronous sinonasal squamous cell carcinoma (SNSCC). Activating EGFR mutations were recently identified in nearly 90% of inverted sinonasal papillomas (ISPs) - a related tumour with distinct morphology. EGFR mutations were, however, not found in OSP, suggesting that different molecular alterations drive the oncogenesis of these tumours. In this study, tissue from 51 cases of OSP and five cases of OSP-associated SNSCC was obtained retrospectively from six institutions. Tissue was also obtained from 50 cases of ISP, 22 cases of ISP-associated SNSCC, ten cases of exophytic sinonasal papilloma (ESP), and 19 cases of SNSCC with no known papilloma association. Using targeted next-generation and conventional Sanger sequencing, we identified KRAS mutations in 51/51 (100%) OSPs and 5/5 (100%) OSP-associated SNSCCs. The somatic nature of KRAS mutations was confirmed in a subset of cases with matched germline DNA, and four matched pairs of OSP and concurrent associated SNSCC had concordant KRAS genotypes. In contrast, KRAS mutations were present in only one (5%) SNSCC with no known papilloma association and none of the ISPs, ISP-associated SNSCCs, or ESPs. This is the first report of somatic KRAS mutations in OSP and OSP-associated SNSCC. The presence of identical mutations in OSP and concurrent associated SNSCC supports the putative role of OSP as a precursor to SNSCC, and the high frequency and specificity of KRAS mutations suggest that OSP and OSP-associated SNSCC are biologically distinct from other similar sinonasal tumours. The identification of KRAS mutations in all studied OSP cases represents an important development in our understanding of the pathogenesis of this disease and may have implications for diagnosis and therapy. Copyright © 2016 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Tumors of the head and neck are extremely diverse and a subset are poorly differentiated and difficult to classify. Recently, a new entity has been described with rhabdoid and/or plasmacytoid cytologic features and a characteristic genetic signature-inactivation of the SMARCB1 (INI-1) tumor suppressor gene. To date, only 16 cases of SMARCB1 (INI-1) deficient sinonasal carcinoma have been described, and there are currently no reports of the cytopathologic features by fine needle aspiration (FNA) cytology. A case of a 77-year-old man who presented with a posterior ethmoid sinus lesion with invasion into the skull base and bone was reported. FNA cytology of a right retropharyngeal lymph node revealed relatively monomorphic, loosely cohesive clusters of plasmacytoid cells with occasional nucleoli, rare intranuclear cytoplasmic inclusions, and mitotic figures in a background of necrosis and absence of overt squamous or glandular differentiation. A diagnosis of metastatic myoepithelial carcinoma was made; however, retrospectively, the surgical excision showed loss of the SMARCB1 (INI-1) tumor suppressor gene by immunohistochemistry. In summary, the cytomorphologic features of SMARCB1 (INI-1) deficient sinonasal carcinoma are relatively nonspecific and overlap with other regional tumors, including myoepithelial neoplasms. As a result, this entity should be considered in the differential diagnosis for a plasmacytoid tumor arising in the sinonasal tract by FNA cytology. Diagn. Cytopathol. 2016;44:700-703. © 2016 Wiley Periodicals, Inc.

Background Sinonasal mucosal melanoma is a rare malignancy with poor prognosis. Methods Patients with sinonasal malignant melanoma who underwent surgery by different approaches were included in this study. Overall survival (OS) and event-free survival were calculated, and statistically significant variables by univariate analysis were entered in a multivariate Cox regression model. Results Pathological staging was pT3, pT4a, and pT4b in 30 cases (51.7%), 17 cases (29.3%), and 11 cases (19.0%). At 3 and 5 years, OS was 43.5% and 29% and event-free survival was 23.6% and 12.4%, respectively. At univariate analysis, OS was significantly influenced by male sex, advanced pT classification, positive margins, and surgical approach; event-free survival was affected by positive margins. At multivariate analysis, the risk of death was independently associated with male sex (hazard ratio [HR] = 2.27; p = .04) and positive margins (HR = 2.32; p = .03). Conclusion Male sex and positive margins were negative prognostic factors. Endoscopic resection did not show an increased risk of death compared with more extensive surgical approaches. © 2015 Wiley Periodicals, Inc. Head Neck 38: E1737–E1745, 2016

Rhabdoid tumors are a spectrum of neoplasias composed of cells which show rhabdoid morphology but are devoid of skeletal muscle differentiation. These tumors are characterized by inactivation of the INI1/SMARCB1 gene and they have been described in virtually every anatomical site, including the central nervous system (CNS) and sinonasal tract. Rhabdoid tumor of the CNS was named atypical teratoid rhabdoid tumor (ATRT) and it mainly affects children under the age of 3 years with supra- or infra-tentorial location.Herein we report the first case of ATRT infiltrating the nasal cavities and skull base in an adolescent. Due to its unusual location, differential diagnosis was challenging and included several other entities such as sinonasal carcinoma or meningioma. Awareness that ATRT may infiltrate the nasal tract and knowledge of its clinico-pathological, immunohistochemical and biomolecular features are essential for its distinction from other rhabdoid tumors which more frequently involve this anatomical site and for appropriate therapeutic management.

Chronic rhinosinusitis (CRS) is a common disease entity with symptoms that may extend beyond the sinonasal tract. Limited data exist regarding the relationship between CRS and the vestibular system, and no previous study has investigated the association between objective vestibular findings on videonystagmography (VNG) and the diagnosis of CRS. We analyzed a prospective database of 3078 patients who underwent VNG at our institution over an 8-year period, which included 70 subjects who had a diagnosis of CRS assigned by an otolaryngologist. Overall, the VNG findings for patients with CRS were similar to those of the general population, with 50% exhibiting normal vestibular function. Peripheral lesions were the most common abnormal VNG finding, with a wide range of subjective symptom descriptions. This preliminary report of the prevalence of objective vestibular findings in patients with CRS may form the basis for future study.

Objectives/Hypothesis Sinonasal malignancies vary in behavior according to histology and anatomical location. Incidence, survival, and optimal treatment for these lesions are thus uncertain in various cases. Our objective was to utilize a national population-based registry to identify the most common sinonasal histopathologies by anatomical site, and subsequently analyze the data by incidence trends, survival rates, patient demographics, and treatment modalities. Study Design Retrospective analysis of the United States National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) registry. Methods The SEER database was examined for patients diagnosed with sinonasal malignancies between 1973 and 2011. Data were stratified according to anatomical site, incidence, survival, histology, staging, and patient demographics. Therapy-based outcomes were analyzed for cases from 1983 to 2011. Results A total of 13,295 patients were identified, with an incidence of 0.83 per 100,000 people. Males comprised 58.6% of cases. Whites represented 81.5% of cases, while blacks comprised 8.7%. Squamous cell carcinoma was the most common histology (41.9%) across all sites of the sinonasal tract. The most common anatomical site of malignancy was the nasal cavity (45.7%), and least common was the frontal sinus (1.2%). For single sites, 5-year disease-specific survival (DSS) was highest for nasal cavity tumors (67.1%) and lowest for overlapping sinus malignancies (37.6%). The overall 5-year DSS for all sinonasal malignancies was 53.7%. Conclusion Sinonasal malignancies are rare entities with poor overall prognosis. By anatomical site, prognosis is best for nasal cavity cancers and worst for overlapping lesions. Level of Evidence 4. Laryngoscope, 125:2491–2497, 2015

Background Local recurrence, and regional and distant metastases, respectively, develop in 30% and 15% of patients with esthesioneuroblastoma (ENB). Methods This case report includes 3 patients with unusual recurrences of ENB in the sinonasal tract. Results Patient 1 developed ENB in the bilateral maxillary sinuses after unilateral endoscopic resection of a left ENB followed by postoperative proton radiotherapy. Patient 2 developed ENB at the left optic-carotid recess 7 years after a craniofacial resection and postoperative radiotherapy. Patient 3 developed ENB over the left zygomatic process of the maxilla 4 years after a cranioendoscopic resection and postoperative radiation therapy for ENB of the ethmoid sinuses with intracranial extension. The possibility of venous tumor emboli must be considered as a likely etiology. However, alternative explanations include the development of a second primary or potential transformation because of tumor signaling. Conclusion We present 3 cases that may represent metastasis of ENB within the sinonasal tract. © 2015 Wiley Periodicals, Inc. Head Neck, 2015

Background Most series about endoscopic resection of adenocarcinomas of the sinonasal tract present outcome data from a small heterogeneous group of patients with a relatively short follow-up period and a wide variety of histological subtypes and treatment protocols. This relatively large study with a very homogeneous study population updates our experience with a stable treatment protocol looking at survival rates, surgical technique, and prognostic factors. Methods We conducted a retrospective analysis of the medical records of 123 patients with adenocarcinoma of the intestinal type primarily treated with endoscopic resection and postoperative radiotherapy (RT) in a single tertiary referral center during the period 1992 to 2010. Results Mean follow-up was 66 months and median follow-up was 54 months. Mean and median follow-up of the subgroup of patients alive at the end of follow-up was 74 months and 61 months, respectively. At 5-years of follow-up, overall survival (OS), disease-specific survival (DSS), and recurrence-free survival (RFS) were 68%, 82%, and 62% respectively. At 10-year follow-up, these numbers were 51%, 74%, and 45%, respectively. Prognostic factors for treatment outcome are predominantly local recurrence, development of distant metastasis, T classification, histopathological classification, and the center where the first surgery was performed. Conclusion This large study of sinonasal adenocarcinoma primarily treated with endoscopic resection and RT confirms that this approach results in good oncologic and functional outcome. © 2015 Wiley Periodicals, Inc. Head Neck 38: 294–300, 2016

Background Pleomorphic adenoma (PA) is the most common benign neoplasm of the major salivary glands. PA of the paranasal sinuses and skull base is an extremely rare pathologic entity. We report 3 cases of sinonasal and skull-base PA (including locoregionally advanced parotid disease) and review other cases in the literature, with special attention to clinical presentation, diagnosis, and management. Methods Retrospective review of patients with PA of the sinuses and skull base treated at 2 tertiary academic medical centers between January 1, 1993 and July 1, 2014. The MEDLINE database was additionally searched for all case series or reports of sinonasal or skull-base PA. Results A total of 32 articles were identified for review. In combination with the authors’ cases, a total of 39 patients were found to have sinonasal and/or skull-base PA. The most common primary sites were the nasal septum and lateral nasal wall. Endoscopic resection was employed in 66% of cases, which achieved oncologic control in more than 80% of cases at a mean follow-up of 2 years. Conclusion PA of the sinonasal tract and skull base is a rare, benign tumor that often may be surgically accessible through an endonasal, endoscopic approach. Recurrent disease may behave more aggressively than PA found in the major salivary glands. Accurate tumor sampling is essential for surgical planning, because malignant transformation does occur. In certain cases of unresectable tumors, endoscopic debulking may improve patient symptoms and quality of life.

Background: Respiratory epithelial adenomatoid hamartomas (REAHs) are rare benign tumors and may manifest as either isolated lesions or in association with sinonasal polyposis. The aim of this study is to report our experience in the management of patients with REAH and to analyze the long-term results of the endoscopic endonasal approach. Methods: A retrospective analysis of a database dedicated to patients with REAH treated between May 2003 and December 2012 was performed. Clinical presentation, demographic, histologic and radiographic features, operative findings, and follow-up data were examined. Results: Twenty-seven patients with REAH, 14 males and 13 females, with a mean age of 51 years, underwent endoscopic sinus surgery (ESS). The most frequent reported symptoms were nasal obstruction (80%), headache (12%), mucous rhinorrhea (20%), and hyposmia (40%). Seventeen cases (first group) were present as isolated masses, 10 cases (second group) were associated with nasal polyposis. The first group with a preoperative diagnosis of REAH was sub

Background Salivary duct carcinoma is a rare, aggressive tumor entity first described by Kleinsasser et al[1] in 1968, as a group of malignant salivary gland tumors characterized by duct formation and central necrosis. A case with an unusual localization in the nasal cavity and maxillary sinus is being described in our report. Methods A 60-year-old patient presented with difficulty of nasal breathing and recurrent episodes of epistaxis from the left nasal cavity over the previous 3 months. On endoscopy, a vulnerable lesion in the left inferior nasal meatus could be seen. Biopsy showed salivary duct carcinoma. Treatment consisted of tumor resection by means of a combined endoscopic-osteoplastic surgical approach to the maxillary sinus, bilateral neck dissection, partial parotidectomy, and adjuvant radiochemotherapy. Results The patient is 6 months after completion of treatment and is free of disease. Conclusion As salivary duct carcinoma of the sinonasal tract is extremely rare, no therapeutic standards have been developed and treatment essentially is based upon experience with ductal adenocarcinoma of the salivary glands. Surgery therapy should be radical, aiming at eradicating locoregional disease with the role of adjuvant radiotherapy lying in the management of cases with negative prognostic factors. © 2016 Wiley Periodicals, Inc. Head Neck, 2016

Objective The aim of this study was to determine the frequency of primary sinonasal adult sarcoma, identify histological subtypes, and analyze prognostic factors. Study Design Retrospective review. Method Forty-eight adult sinonasal sarcomas included in the French Sarcoma Group database (Conticabase) were reviewed. Results The most frequent tumor types were alveolar rhabdomyosarcoma (33.3%), embryonal rhabdomyosarcoma (14,6%), unclassified sarcoma (14.6%), and leiomyosarcoma (12.5%). All round cell tumors were rhabdomyosarcomas. The 5-year overall survival (OS), metastasis-free survival (MFS), and local recurrence-free survival (LRFS) rates were 62.3%, 73%, and 88.8%, respectively. Histotype was a prognostic factor for OS, MFS, and LRFS, with the worst prognosis associated with rhabdomyosarcomas, regardless of the subtype. The tumor grade influenced the OS and MFS. Surgery was a predictive factor for a complete response. Conclusions These results suggest that sinonasal tract should be considered as an unfavorable site for rhabdomyosarcoma. Moreover, surgery should always be considered in treatment. Level of Evidence N/A. Laryngoscope, 125:615–623, 2015

Background Seromucinous hamartoma, a type of epithelial hamartoma, is a rare benign glandular proliferation of the sinonasal tract and nasopharynx. Herein, we present 2 rare cases of seromucinous hamartoma arising in the nasal cavity medial to the middle turbinate. Methods and Results This is a case report of 2 patients diagnosed with seromucinous hamartoma of the nasal cavity and a review of the literature. Conclusion Seromucinous hamartoma of the nasal cavity is an exceedingly rare diagnosis but should be included in the differential diagnosis of a posterior nasal tumor. Most cases arise from the posterior nasal cavity medial to the middle turbinate rather than lateral to the middle turbinate. © 2014 Wiley Periodicals, Inc. Head Neck 37: E15-E18, 2015

Background Trefoil factor family (TFF) peptides are mucin-associated secretory products that are produced in the airways and gastrointestinal tract. These peptides appear to play an important role in mucosal healing and epithelial protection and are overexpressed in chronically inflamed gastrointestinal tissues. We hypothesize that TFF peptides may also be differentially expressed in the sinonasal tissue of patients with and without chronic rhinosinusitis (CRS). Methods Ethmoid sinus tissue was obtained from patients with CRS without nasal polyps (CRSsNP) (n = 12), CRS with nasal polyps (CRSwNP) (n = 12), and nondiseased controls (n = 7). Messenger RNA (mRNA) and protein were extracted from samples and expression of TFF1, TFF2, and TFF3 were assessed using quantitative real-time polymerase chain reaction qRT-PCR and Western blots, respectively. Tissue localization of TFF expression was confirmed using immunohistochemistry. Results TFF1 and TFF3 were both highly expressed in sinonasal tissue, while TFF2 was expressed at near-undetectable levels. CRSsNP tissue had a statistically significant increase in the expression of both TFF1 and TFF3. No difference in TFF expression was found between control and CRSwNP patients. Conclusion TFF1 and TFF3 are overexpressed in CRSsNP. The role of TFF peptides in mucosal protection and repair suggests a possible important physiologic role in maintaining the sinonasal epithelial barrier and modulating innate immunity in the sinonasal tract.

Background Sinonasal localization of Ewing's sarcoma in adults is an exceedingly rare event. Methods The clinical records of 5 patients with primary sinonasal Ewing's sarcoma treated from 1992 to 2012 were retrospectively analyzed. All pathologic slides were reviewed by 2 experienced pathologists. All patients underwent multimodality treatments. Results Median age was 36 years (range, 25–52 years). At referral, 2 patients had the original diagnosis changed by review of the histologic slides. Tumors were classified as T4aN0M0 (4 patients) and T2N0M0 (1 patient). Median follow-up was 110 months (range, 70–139 months). Only 1 patient, who started treatment elsewhere based on an incorrect histologic diagnosis, experienced multiple recurrences and eventually died of widespread metastasis. Conclusion Correct pathologic diagnosis can have a crucial impact on treatment planning and outcome. Multimodality therapy is the key for long-term successful results. Because of the rarity of the tumor, referral to highly experienced care centers is strongly recommended. © 2016 Wiley Periodicals, Inc. Head Neck 39: E45–E50, 2017

Background Sinonasal melanoma (SNM) is a rare malignancy that commonly presents at an advanced age and has a slight male predominance. Local recurrence has been implicated as a major reason for treatment failure, and there are poor reported 5-year survival rates. We analyzed the impact of specific location within the sinonasal region on the survival of this rare malignancy. Methods The U.S. National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) registry was used to extract data on SNM between 1973 and 2009. Survival trends and hazard ratios (HRs) were calculated to compare the prognostic implications of involvement of varying areas of the sinonasal tract. Results A total of 567 cases were identified. Females constituted 56.44% patient. Disease-specific survival (DSS) at 5 years was 36.66% for patients diagnosed with nasal cavity disease, 23.80% for patients with maxillary sinus tumors, and 18.20% for patients with ethmoid sinus disease. Patients showing evidence of overlapping sinus involvement had approximate 1-year survival of 54.45% and none survived beyond 49 months. HRs for maxillary sinus, ethmoid sinus, and overlapping sinus disease were 1.34, 1.60, and 2.30, respectively. All DSSs and HRs were statistically significant (p < 0.05). There was a higher proportion of earlier-stage disease in the nasal cavity compared to the most common paranasal sinus region (p < 0.05). Conclusion Prognosis in SNM is dependent on the anatomic subsite. Paranasal sinus involvement indicates a poorer prognosis when compared to nasal cavity disease. Patients presenting with overlapping sinus involvement have the poorest prognosis.

Background The purpose of this study was to assess the prognostic value of the 3 staging systems found in the literature for sinonasal mucosal melanomas tumors: the Ballantyne staging system modified by Prasad (Ballantyne/Prasad staging system), the American Joint Committee on Cancer (AJCC) TNM classification for mucosal melanomas (mmTNM), and the 2009 AJCC TNM classification for carcinomas of the nasal cavity and sinuses (carTNM). Methods A retrospective study of 35 patients treated between 1995 and 2010 was conducted for this study. Each patient was retrospectively staged using the Ballantyne/Prasad staging system, mmTNM, and carTNM. Results There were 20 women (57.1%) and 15 men (42.9%). Only carTNM was significantly correlated with overall survival (p = .012) and disease-free survival (p = .041). The other 2 classifications were not correlated with survival except for metastatic patients whose overall survival was lower (p = .032). Conclusion On the basis of these findings, we believe that carTNM should be the primary staging system for patients with mucosal melanomas of the sinonasal tract. © 2013 Wiley Periodicals, Inc. Head Neck 36: 311–316, 2014

Background Hemangiopericytomas are typically found in soft-tissue, but only rarely found in the sinonasal tract. Sinonasal hemangiopericytomas have been reported mostly through case studies, and optimal treatment is considered surgical excision. Classically, open surgical methods of tumor extirpation have been considered standard of care. With the wider use of endoscopic methods, an updated systematic review in terms of treatment is warranted. Methods Cases were identified using a MEDLINE and PubMed search. Relevant studies were identified, and data was extracted regarding patient demographics, presenting symptoms, tumor characteristics, treatment, and outcomes. Results A total of 128 cases were collected from 56 articles, consisting of case reports and series. The most common presenting symptoms were epistaxis, nasal obstruction, and facial pain/swelling/pressure. Computed tomography (CT) and X-ray were the most common modes of imaging during diagnosis and operative planning. The tumor often occupied multiple locations in the sinonasal tract at initial presentation. Surgical resection was the mainstay of treatment in 126 of the 128 cases (98.4%), either through open resection or endoscopic techniques. Surgical removal resulted in no recurrence in 79.7% of the cases. The use of endoscopic techniques increased significantly in the past decade. This review found no significant difference in terms of recurrence between endoscopic and open treatment groups, age, gender, and unilocality vs multilocality of tumor. Conclusion Surgical management remains the mainstay of treatment for hemangiopericytomas. Endoscopic resection of these lesions has increased over the last few decades and has become a safe, viable, and reasonable alternative to open resection.

Franchi A, Palomba A & Cardesa A (2011) Histopathology 59, 1034–1045 Current diagnostic strategies for undifferentiated tumours of the nasal cavities and paranasal sinuses Several malignant tumours occurring in the sinonasal tract may present with an undifferentiated morphology. Overall, these lesions pose significant diagnostic difficulties for the surgical pathologist, especially in limited biopsy material, but their correct classification is becoming increasingly important for an appropriate treatment strategy. This review deals with the criteria for differential diagnosis of these neoplasms, with emphasis on recent advances in immunohistochemistry and molecular biology, as well as with previous progress in electron microscopy. Through careful microscopic examination of haematoxylin and eosin-stained sections, in the light of clinical information and imaging data, a list of differential diagnoses can be made and an appropriate panel of antibodies can be chosen to further categorize the tumour. An initial panel including cytokeratins, synaptophysin, S100 protein, desmin and CD45 may allow the classification of most lesions or may help to narrow the list of differential diagnoses. Further refinement can be obtained through second-line markers, including in-situ hybridization for Epstein–Barr virus, other neuroendocrine markers, melanocytic markers, myogenin, CD99, other lymphocyte markers, and CD138 and light chains. Finally, molecular analysis can further assist in the recognition of specific entities such as nuclear protein in testis midline carcinoma, Ewing’s sarcoma/peripheral neuroectodermal tumour, alveolar rhadbomyosarcoma, and poorly differentiated synovial sarcoma.

To determine whether variations in gene expression exist at multiple subsites along the sinonasal tract in patients with chronic sinusitis with polyps and in healthy controls.Prospective, controlled study.Academic medical center.Tissue expression levels of 5 genes, previously found to be characteristic of ethmoid polyps, were measured using real-time quantitative polymerase chain reaction in 100 sinonasal tissue samples. Specimens harvested from 5 regions--the ethmoid sinus, septum, inferior turbinate, middle turbinate, and lateral nasal wall--in 10 patients with chronic sinusitis and ethmoid polyps were compared to tissue from similar regions in 10 control patients without sinusitis. Western blot analysis was performed to validate differential gene expression at the protein level.Gene expression levels of ethmoid polyps differed significantly from those of healthy ethmoid mucosa, as well as tissue from 4 surrounding anatomical sites in both patients with chronic sinusitis and controls. Alterations specific to the polyp tissue included downregulated genes, prolactin-induced protein (fold change 377.2 ± 169.0, P.0001), and zinc α2-glycoprotein (fold change 72.1 ± 26.5, P.0001), as well as upregulated genes, met proto-oncogene (fold change 2.5 ± 0.7, P = .029), and periostin (fold change 7.5 ± 3.4, P = .003). No significant differences in gene expression was found for neurabin 2 (fold change 1.0, P = .99).The transcriptional pattern of ethmoid polyps appears to be unique compared with other subsites in the sinonasal cavity of patients with chronic sinusitis. Care must be taken when collecting specimens for molecular studies of the sinonasal tract to differentiate polyp from nonpolyp tissue in chronic sinusitis.

Sinonasal undifferentiated carcinoma (SNUC) is an aggressive malignancy of disputed histogenesis that arises in the sinonasal tract and has an extremely poor prognosis. Despite multimodality treatment with surgical resection, radiation, and chemotherapy, recurrence is common. The tumor spreads by direct local extension, but also metastasizes to lymph nodes, brain, lung and bone. To date, reports of metastasis to the skin have not been published. We report a case of a 58-year-old woman diagnosed with SNUC who underwent surgical resection of the tumor followed by chemoradiation. The tumor soon recurred, and she required wide re-excision. Two months after this procedure, she developed multiple dermal nodules in the head and neck region, clinically suspicious for metastases. Biopsy of a nodule from the right neck revealed a poorly differentiated carcinoma, with morphological and immunohistochemical findings consistent with a metastasis of the patient's known SNUC. We conclude that the skin may be a rare site of metastasis of SNUC, and in some cases may be the presenting sign of tumor recurrence despite aggressive multimodality treatment.

BACKGROUND: The role of human papillomavirus (HPV) in the pathogenesis of squamous cell carcinomas (SCCs) of the sinonasal tract and its clinicopathological implications were evaluated. METHODS: All SCCs of the sinonasal tract diagnosed in the Hospital Clinic of Barcelona from 1981 to 2006 were retrospectively evaluated (N = 60). Clinical and pathological data were reviewed. HPV infection was determined and typed by amplification of HPV DNA by polymerase chain reaction using the SPF-10 primers. p16INK4a expression was determined by immunohistochemistry. Overall and progression-free survival for HPV-positive and -negative patients was estimated by Kaplan-Meier analysis and by the use of a multivariate Cox proportional hazards model. RESULTS: HPV DNA was detected in tumor tissue of 12 of 60 (20%) patients. HPV16 was identified in 11 tumors and HPV35 in 1. Immunohistochemistry for p16INK4a stained all HPV-positive and no HPV-negative tumors (P < .001). No differences were observed in terms of site and histological grade or stage at presentation between HPV-positive and -negative tumors. However, HPV-positive patients had a significantly better 5-year progression-free survival (62%; 95% confidence interval [CI], 23%-86% vs 20%; 95% CI, 9%-34%; P = .0043, log-rank test) and overall survival (80%; 95% CI, 20%-96% vs 31%; 95% CI, 15%-47%; P = .036, log-rank test) than patients with HPV-negative tumors. In multivariate analysis, HPV-positive tumors were associated with improved progression-free survival (hazard ratio, 0.21; 95% CI, 0.17-0.98; P = .012). CONCLUSIONS: A subgroup of sinonasal SCCs is associated with HPV infection. These tumors have a significantly better prognosis. Cancer 2009. © 2009 American Cancer Society.

Background. Head and neck solitary extramedul- lary plasmacytomas are rare plasma cell neoplasms. Literature review reveals only small numbers of reports. The objective was to review our experience with treating patients with this neo- plasm. Methods. A retrospective chart review between 1960 and 2000 was performed. Sixty-eight patients with head and neck extramedullary plasmacytomas were included. Results. The sinonasal tract was the most common site. Thirty-nine patients were treated with radiation, 8 with surgery, 14 with surgery and radiation, and 3 with chemoradiation. Me- dian follow-up was 8 years. The 5-year local recurrence-free rate (LRFR) was 81%. There was a trend toward improved LRFR in patients treated primarily with radiation. Regional recurrence at 5 years was 5%. Multiple myeloma developed in 23% of patients. The 5-year survival was 76%. Conclusions. Radiation is the treatment of choice with sur- gery reserved for large tumors and extensive bone destruction. Long-term follow-up is essential because local recurrence and disseminated disease can occur many years postdiagnosis. V C 2008 Wiley Periodicals, Inc. Head Neck 30: 1012-1019, 2008

Objectives/Hypothesis: Neuroendocrine carcinoma (NEC) of the sinonasal tract is rare. In this present study, we report our treatment experience with sinonasal NEC. Study Design: Retrospective case series. Methods: A retrospective review of the clinical outcomes and pathology of 18 patients with NEC arising from the sinonasal region. Results: Ten tumors were primary NEC without previous radiation, and eight tumors were postirradiated NEC occurring within the radiation field for previous nasopharyngeal carcinoma in six patients and tonsillar lymphoma in one and neck metastasis of unknown primary origin in one, with an interval between previous radiotherapy and diagnosis of NEC from 82 to 385 months, with a mean of 197 months. Fifteen tumors were small cell carcinoma, two were atypical carcinoid, and one was typical carcinoid tumor. Fifteen patients underwent surgery with/without postoperative adjuvant chemoradiotherapy. Three patients received induction chemotherapy or primary radiotherapy with further definitive treatment. The 5-year disease-free and overall survival rates of all 18 patients were 56.1% and 62.2%, respectively. In comparing primary NEC with postirradiated NEC, they were similar in age, sex distribution, stage, pathology, and treatment, and the 5-year overall survival rates were 70% and 62.5%, respectively. Conclusions: In this series, postirradiated NEC is common, which may be the result of there being a large number of long-term nasopharyngeal carcinoma survivors. The prognoses of postirradiated NEC and primary NEC appear to be similar despite the relatively short follow-up period in the postirradiated NEC group.

Background. This study analyzes the management and outcomes of a series of 10 malignant peripheral nerve sheath tumors (MPNST) of the head and neck. Methods. From 1984 to 2004, 10 patients underwent surgical treatment of a MPNST. We retrospectively reviewed presenting symptoms, radiological findings, surgical management, and follow-up status and performed a literature review. Results. Eight tumors were located at the lateral skull base; 2 involved the vagus nerve in isolation. Two lesions were growing within the sinonasal tract. The most common presenting symptom was a rapidly enlarging cervical mass. Seventy percent of the tumors could be resected completely. Long-term follow-up showed a 2-year disease-specific survival rate of 50% and 5-year survival rate of 20%. Negative prognostic indicators were advanced tumor stage, early recurrence, and presumably also the presence of von Recklinghausen's disease. Postoperative adjuvant radiotherapy was found to make no difference in outcome. Conclusions. Although rare, MPNST is one of the most aggressive tumors in the head and neck area. Complete tumor removal is the mainstay of treatment and most important prognostic factor of MPNST. Adjuvant radiotherapy should be used to assist surgical excision in local control. The role of adjuvant chemotherapy remains controversial. © 2006 Wiley Periodicals, Inc. Head Neck, 2007.

Background. Acinic cell carcinoma is a low-grade malignant epithelial salivary gland neoplasm with a predilection for the parotid gland. To date, only 11 cases of sinonasal acinic cell carcinomas have been reported in the English-language literature. We present the clinicopathologic features of four sinonasal acinic cell carcinomas. Methods. The demographic data and pathologic material of four patients with sinonasal acinic cell carcinoma identified from the files of the Department of Pathology at The University of Texas M. D. Anderson Cancer Center between 1984 and 2002 were reviewed. Results. The four patients were two men and two women, with an age range of 42 to 65 years (mean, 54 years). The patients were initially seen with unilateral nasal obstruction. Histologically, all tumors were composed of round to ovoid cells with clear and/or basophilic granular cytoplasm and round, hyperchromatic, small, eccentrically located nuclei. The growth pattern was lobular, solid, and follicular. Histochemically, periodic acid–Schiff diastase-resistant granules were demonstrated in all cases. All patients were treated surgically. In addition, one patient received postoperative radiation. All patients are alive and well, with follow-up from 4 to 17 years. Conclusions. Sinonasal acinic cell carcinoma is a distinct low-grade carcinoma that can be distinguished from other neoplasms by light microscopy and histochemical staining methods. Pathologists and surgeons should be aware of the occurrence of this type of salivary gland neoplasm in the sinonasal tract. © 2005 Wiley Periodicals, Inc. Head Neck27: XXX–XXX, 2005

Objective: To demonstrate the potentials and limitations of three different endoscopic procedures employed for treatment of inverted papilloma (IP) of the sinonasal tract. Study Design: Retrospective analysis of a cohort of patients treated at two University hospitals. Methods: From January 1992 to June 2000, 47 patients with IP underwent endoscopic resection. Preoperative workup included multiple biopsies of the lesion and imaging evaluation by computed tomography or magnetic resonance imaging. Massive skull base erosion, intradural or intraorbital extension, extensive involvement of the frontal sinus, abundant scar tissue caused by previous surgery, or the concomitant presence of squamous cell carcinoma were considered absolute contraindications for a purely endoscopic approach. Three types of resection were used: ethmoidectomy with wide antrostomy and sphenoidotomy (type 1) for IPs confined to the middle meatus, medial maxillectomy with ethmoidectomy and sphenoidotomy (type 2) for IPs partially invading the maxillary sinus, and a Sturmann-Canfield operation (type 3) for IPs involving the mucosa of the alveolar recess or of the anterolateral corner of the maxillary sinus. All patients were followed by periodic endoscopic evaluations. Results: Type 1, 2, and 3 resections were performed in 26, 15, and 6 patients, respectively. No recurrences were observed after a mean follow-up of 55 (range 30–132) months. One patient, who underwent a type 2 resection, developed a stenosis of the lacrimal pathways requiring endoscopic dacryocystorhinostomy. Conclusions: Our experience confirms that endoscopic surgery is an effective and safe method of treatment for most IPs. The availability of different endoscopic techniques allows the entity of the dissection to be modulated in relation to the extent of disease. Strict application of selection criteria, meticulous use of subperiosteal dissection in the involved areas, and regular follow-up evaluation are key elements for success.

Background. Although sinonasal inverted papilloma (IP) is a rare benign tumor, it has a tendency to recur and is sometimes associated with squamous cell carcinoma (SCC). Therefore, postoperative long-term follow-up of these patients is recommended. We previously reported that serum SCC antigen might be a useful tumor marker for sinonasal IP. In this study, we investigated whether serum SCC antigen level has a correlation with disease status and is useful in the early detection of recurrent disease. Methods. Blood samples for the analysis of serum SCC antigen were taken from 28 IP patients before and after surgical treatment. Results. Twenty-five (89%) of 28 cases showed evaluated serum SCC antigen levels above the upper limit. This marker level decreased in all cases after surgical resection. Four of these patients had a recurrence. None of the patients with recurrent tumor showed symptoms at the time of detection of their recurrent tumor, and recurrence was discovered from elevated levels of SCC antigen. Conclusions. Serum SCC antigen level has a correlation with disease status of IP and has a potential to serve as a useful tool for monitoring the course of disease. SCC antigen is a reliable tumor marker in the management of sinonasal IPs. © 2004 Wiley Periodicals, Inc. Head Neck27: 44–48, 2005

Objectives/Hypothesis Mesenchymal chondrosarcoma of the sinonasal tract is a rare, malignant tumor of extraskeletal origin. Isolated cases have been reported in the English literature, with no large series evaluating the clinicopathological aspects of these tumors. Study Design Retrospective review. Methods Thirteen patients with sinonasal mesenchymal chondrosarcoma were retrieved from the Otorhinolaryngologic—Head and Neck Registry of the Armed Forces Institute of Pathology. Results Nine women and 4 men (age range, 11 to 83 y; mean age, 38.8 y) presented with nasal obstruction (n = 8), epistaxis (n = 7), or mass effect (n = 4), or a combination of these. No patients reported prior head and neck irradiation. The maxillary sinus was the most common site of involvement (n = 9), followed by the ethmoid sinuses (n = 7) and the nasal cavity (n = 5). Tumors had an overall mean size of 5.1 cm. Microscopically, the tumors displayed a small, blue, round cell morphology appearance arranged in a hemangiopericytoma-like pattern with foci of cartilaginous matrix. All cases were managed by surgery with adjuvant radiation therapy (n = 4) and/or chemotherapy (n = 3). The overall mean survival was 12.1 years, although five of six patients who developed local recurrences died of disease (mean survival, 6.5 y). Six patients were alive and disease free (mean survival, 17.3 y), and two patients were lost to follow-up. Conclusions Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. The pattern of growth and scarcity of cartilaginous matrix result in frequent misdiagnosis. Recurrence develops in approximately one-third of patients and seems to predict a poor prognosis. Aggressive, exenterative surgery combined with adjuvant therapy appears to yield the best clinical outcome.

BACKGROUND Primary and metastatic malignancies that originate in the sinonasal tract are rare and histologically diverse. The role of fine-needle aspiration biopsy (FNAB) and the cytomorphologic features of these tumors have not been specifically addressed. METHODS The authors reviewed 22 cytology cases (20 FNABs, 1 sputum sample, and 1 pleural fluid sample) from 18 patients with malignancies originating in the sinonasal tract (17 carcinomas, 3 melanomas, and 2 sarcomas) and assessed the cytomorphology, cytohistologic correlation, and ability of cytology to render a specific diagnosis. RESULTS Primary and metastastic sites sampled by FNAB included masses in or around the nose (n = 2), orbit (2), maxillary sinus (2), frontal sinus (1), intraoral area (1), preauricular area (1), soft tissue neck masses, parotid and lymph nodes (10), and cervical spine (1). Exfoliative cytology was positive in two samples of sputum and pleural fluid, representing the initial cancer diagnosis before the sinonasal primary tumor was detected. Seventeen of 22 (77.3%) cases were classified as carcinoma not otherwise specified, carcinoma with specific differentiation, sarcoma, or melanoma. Cytology failed to correctly classify the specific subtype of three carcinomas. The cytologic features that were evaluated included cellularity, cellular arrangement, nuclear features, nuclear-to-cytoplasmic ratio, and the background appearance. CONCLUSIONS Sinonasal tract malignancies demonstrate a wide range of cytologic findings but specific features allowing for an accurate and definitive diagnosis are often present in many tumors. Fine-needle aspiration cytology is an important diagnostic tool in the management of sinonasal malignancies and can be complemented by the use of ancillary studies for the diagnosis of poorly differentiated or nonepithelial tumors. Cancer (Cancer Cytopathol) 2003;99:105–12. © 2003 American Cancer Society.

Background Eosinophilic angiocentric fibrosis (EAF) is a rare fibroinflammatory lesion of the sinonasal tract that occurs mainly in young to middle-aged female patients. Only two previous cases affecting male patients have been reported, and its etiopathogenesis remains unknown. The authors report on the third case of the entity in a male patient and review the 12 previously reported cases. Case Report A 52-year-old male patient was initially seen with a 15 years history of allergic rhinitis, progressive nasal obstruction, and left-sided hearing loss. All laboratory tests were unremarkable, except the nasal discharge eosinophil count that showed a conspicuous eosinophilia. The video-assisted-nasofibroscopic examination and CT scans disclosed a thickened deviated nasal septum with a subjacent infiltrative lesion. The histologic analysis of the nasal septum showed a variable mixed inflammatory cellular infiltration mainly composed of eosinophils, plasma cells, and histiocytes with a perivascular distribution; in other areas, an angiocentric fibrosing lesion with a peculiar perivascular onion-skin pattern was observed. The patient had a partial resection of the lesion with symptomatic control. Conclusions The presence of rhinitis and nasal eosinophilia in our case associated with the clinical aspects of the previously reported cases further support an allergic cause for EAF. © 2002 Wiley Periodicals, Inc. Head Neck 24: 307–311, 2002; DOI 10.1002/hed.10041