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1.  Sinonasal Neoplasia – Clinicopathological Profile And Importance of Computed Tomography 
Background
Nasal cavity and Paranasal sinus malignancies are very rare, in which maxillary sinus is the commonest, followed by ethmoid, frontal and sphenoid sinus. Computed Tomography (CT) & Magnetic Resonance Imaging (MRI) play a key role in diagnosis, staging and management of paranasal sinuses and nasal pathologies. Multiplanar imaging in CT helps better imaging of critical anatomical areas. Aim of our study was to study the incidence, clinical features, CT features and its importance in the management of sinonasal neoplasms.
Materials and Methods
This prospective study was carried out in a tertiary care hospital of MP, India. Consecutive 40 histologically proven cases of sinonasal neoplasia who visited the Department of Otorhinolaryngology and Radiotherapy are included in our study. Demography and clinical features were recorded. Cases of nasal and paranasal sinus masses diagnosed on CT attending ENT and Radiotherapy OPD or admitted in the Radiotherapy ward forms the material of this study. This included patients of both sexes and all ages. Histopathological examination was asked to confirm the diagnosis made on CT.
Results
There were total 40 cases of sinonasal neoplasia among which 24 were benign. Almost all the benign cases were seen in the age group <40 y with mean age of 20 y and most of the malignant cases were seen in the age group above 40 y with mean age of 55 y. In our study we found male preponderance with male female ratio of 4:1 in both benign and malignant conditions. The commonest presenting symptoms of the patients with sinonasal masses in our study was nasal obstruction (75%) and nasal discharge (67.5%) followed by nasal mass (65%), epistaxis (62.5%) and headache (60%). Angiofibroma and papilloma were the commonest benign lesions. Commonest malignant lesion was squamous cell carcinoma. Of the malignant Sinonasal tumours studied in our series, maxillary sinus was involved in 13, ethmoid sinuses and nasal cavity in 10 cases each, and frontal sinuses in 2 cases.
Conclusion
Contrast enhanced computed tomography with multiplanar reconstruction is an excellent modality for imaging sinonasal masses. CT can define the character of the sinonasal mass, thus differentiating benign from malignant. CT helps better imaging of critical anatomical areas and helpful in planning treatment procedures such as surgery and radiotherapy.
doi:10.7860/JCDR/2015/13514.6026
PMCID: PMC4525572  PMID: 26266182
Benign; Malignant;  Paranasal sinuses
2.  Sinonasal Malignancies: Long Term Follow Up After Surgical Management—An Analysis of Outcomes 
Sinonasal malignancies are rare and survival analysis in affected patients is arduous and perplexing due to various factors. In this review article, attempt has been made to overcome some of those factors while analysing survival outcomes. Aims and objectives: The aim of this study was to share the experience of a tertiary care centre in the surgical management of sinonasal malignancies over 12 years. Materials and Methods: This study is a retrospective chart review, and in this study, hospital records of 58 patients with biopsy proven sinonasal malignancies were studied. Only the patients undergoing primary or salvage surgery at our institution from May-2000 to April-2012 with a minimum follow up of 2 years were included. Statistical analyses such as means, proportions, Kaplan–Meier analysis and Cox's regression model were done. Results: Majority of the patients were males (n = 43) belonging to fourth and fifth decades. Squamous cell carcinoma was the most common (n = 17) histopathological type, followed by adenoid cystic carcinoma (n = 14). Majority presented with stage IV disease (n = 42). 17 patients were operated for recurrent disease. Over all 5 year survival was 72 % and disease free survival was 44 %. Separate analysis for epithelial and matched non-epithelial group showed poorer prognosis with epithelial group (p = 0.0120). Multivariate analysis showed histopathological type (epithelial) and presence of pathological risk factors (positive margins and/or perineural invasion) affecting survival. Conclusions: Advanced stage presentation is the norm for sinonasal malignancies. This study noted a 5 year overall survival of 72 % and disease free survival of 44 %. Epithelial histopathology carries poorer prognosis then other counterparts and incomplete removal is shown to add to poor prognosis independently.
doi:10.1007/s12070-014-0742-2
PMCID: PMC4298580  PMID: 25621228
Sinonasal malignancies; Squamous cell carcinoma; Survival analysis; Long term outcomes
3.  Factors Contributing to Poor Management Outcome of Sinonasal Malignancies in South-West Nigeria 
Ghana Medical Journal  2013;47(1):10-15.
Summary
Objective
To describe the clinico-pathologic manifestations of sinonasal malignancies, identify the contributing factors to delay in presentation and recommend ways of preventing them in a resource challenged environment.
Design
A questionnaire based cross sectional descriptive study of patients with sinonasal malignancies between 2006 and 2011.
Setting
Hospital based study at the Otorhinolaryngology Department, University College Hospital, Ibadan. Participants: 61 patients diagnosed with sinonasal malignancies
Main outcome measures
Patients demographic and essential medical data were collected with a structured, interviewer assisted questionnaire and results analysed using descriptive statistics.
Results
There were 28(45.9%) males and 33(54.I %) females; mean age 37years. The common presentations were epistaxis, nasal obstruction and facial asymmetry and 96.7% patients with squamous cell carcinoma presented in advanced disease stage (Stage 3 & 4). Over 47% patients presented a year after onset of symptoms. Factors which included self-medication, wrong advice from relations/friends to consult traditional herbalist or quacks for treatment and traditional & religious beliefs contributed to delay in presentation to hospitals. High cost of medical treatment, unwelcoming attitudes of some hospital staff, lack of confidence in orthodox medicine and proximity to health facilities were reasons given for not considering hospital as the first place to seek medical treatment.
Conclusion
Health education to create awareness of sinonasal malignancies and provision of affordable and accessible health facilities especially in rural areas are recommended ways to encourage patients to present early in hospitals. This will improve the management outcome and quality of life of patients with sinonasal malignancies.
PMCID: PMC3645191  PMID: 23661850
Delayed presentation; Health care services; Outcome; Sinonasal malignancies
4.  Imaging of sinonasal tumours 
Cancer Imaging  2012;136-152.
Abstract
More than 70 benign and malignant sinonasal tumours and tumour-like conditions have been described. However, sinonasal tumours are rare, and sinonasal cancers comprise only 3% of all head and neck cancers and 1% of all malignancies, with a peak incidence in the 5th to 7th decades and with a male preponderance. The early symptoms and imaging findings of sinonasal tumours are similar to rhinosinusitis with runny and stuffy nose, lacrimation and epistaxis and therefore neglected both by the patients and doctors. When late symptoms such as anosmia, visual disturbances, cranial neuropathy (Cn II, IV, V, VI) or facial swelling appear, the patient is referred to sinonasal endoscopy or imaging. At the time of correct diagnosis more than half of the tumours have reached an advanced stage with a poor prognostic outcome. Even if imaging is performed in the early stages, a radiologist inexperienced with sinonasal anatomy and tumour features may easily interpret early signs of a malignant tumour as rhinosinusitis or a lesion that does not require follow-up. This article presents the imaging findings in some of the most common benign and malignant sinonasal tumours, and the TNM classification and staging of sinonasal carcinomas.
doi:10.1102/1470-7330.2012.0015
PMCID: PMC3362868  PMID: 22571851
Head; neck; sinonasal; neoplasms; computed tomography; magnetic resonance imaging; tumour staging
5.  Endonasal Endoscopic Surgery in the Management of Sinonasal and Anterior Skull Base Malignancies 
Head and Neck Pathology  2016;10(1):13-22.
Sinonasal malignancies represent a rare subset of tumors with a wide variety of histopathologic diagnoses and overall poor prognosis. These tumors tend to have an insidious onset with non-specific symptoms which often leads to delayed diagnosis and advanced local disease at presentation. The principal goal of surgery is to obtain a negative margin resection. Open craniofacial techniques are well established in the management of sinonasal malignancies and remain the treatment of choice for many advanced tumors. Over the past couple of decades, there has been tremendous application of endoscopic techniques to skull base pathologies including sinonasal malignancies. For selected cases, endonasal endoscopic techniques can be performed with curative intent and reduced surgical morbidity and mortality. Here we discuss principles of surgical management of sinonasal malignancies, review the techniques of endonasal endoscopic resection of sinonasal malignancies, and highlight the importance of pathology in the multi-disciplinary management of patients with these complex lesions.
doi:10.1007/s12105-016-0687-8
PMCID: PMC4746133  PMID: 26830407
Endoscopic; Expanded endonasal approach; Paranasal sinuses; Sinonasal malignancy; Skull base
6.  Sinonasal Tract Malignancies: Prognostic Factors and Surgery Outcomes 
Background:
Cancers of the sinonasal region are rare and its survival rate remains poor because most of the patients are asymptomatic and diagnosed in advanced stages with surrounding important structures.
Objectives:
This study attempted to analyze the clinical and histological features in addition to survival and prognostic factors of surgical treatment of sinonasal cancers.
Patients and Methods:
A retrospective cohort study, involving 36 patients with sinonasal cancer who were treated with surgery in our hospital between 2000 and 2010, was performed. Patients were selected based on the convenience sampling. Patients treated with radiotherapy and/or chemotherapy were excluded from the analysis. Clinical symptoms and histologic findings of patients as well as malignant tumor staging and its prognosis were collected from archives.
Results:
We found that overall 3 and 5-year survival rates of subjects were 52.8%, and 41.6%, respectively. There was a negative correlation between the clinical stage and survival. There was a significant difference between infrastructural and suprastructural localization in 5-year survival rate (P = 0.018). In the present study, there was a strong relationship between the local control and overall survival (P < 0.01). Overall 5-year survival rate was similar in patients both in the exenterated orbit and preserved orbit (P > 0.05).
Conclusions:
The present study has demonstrated that clinical stage, suprastructural tumor, and the presence of tumor- positive resection margins are the most significant prognostic factors affecting local tumor control and survival. As a result of this study, these tumors should be treated in early stages by surgical margin of resection followed by adjuvant radiotherapy.
doi:10.5812/ircmj.14118
PMCID: PMC3955510  PMID: 24693395
Paranasal Sinus Neoplasms; Surgery; Survival Rates; Prognosis
7.  Sinonasal Tract and Nasopharyngeal Adenoid Cystic Carcinoma: A Clinicopathologic and Immunophenotypic Study of 86 Cases 
Head and Neck Pathology  2013;8(1):88-109.
Primary sinonasal tract and nasopharyngeal adenoid cystic carcinomas (STACC) are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. Eighty-six cases of STACC included 45 females and 41 males, aged 12–91 years (mean 54.4 years). Patients presented most frequently with obstructive symptoms (n = 54), followed by epistaxis (n = 23), auditory symptoms (n = 12), nerve symptoms (n = 11), nasal discharge (n = 11), and/or visual symptoms (n = 10), present for a mean of 18.2 months. The tumors involved the nasal cavity alone (n = 25), nasopharynx alone (n = 13), maxillary sinus alone (n = 4), or a combination of the nasal cavity and paranasal sinuses (n = 44), with a mean size of 3.7 cm. Patients presented equally between low and high stage disease: stage I and II (n = 42) or stage III and IV (n = 44) disease. Histologically, the tumors were invasive (bone: n = 66; neural: n = 47; lymphovascular: n = 33), composed of a variety of growth patterns, including cribriform (n = 33), tubular (n = 16), and solid (n = 9), although frequently a combination of these patterns was seen within a single tumor. Pleomorphism was mild with an intermediate N:C ratio in cells containing hyperchromatic nuclei. Reduplicated basement membrane and glycosaminoglycan material was commonly seen. Necrosis (n = 16) and atypical mitotic figures (n = 11) were infrequently present. Pleomorphic adenoma was present in 9 cases; de-differentiation was seen in two patients. Immunohistochemical studies showed positive reactions for pan-cytokeratin, CK7, CK5/6, CAM5.2, and EMA, with myoepithelial reactivity with SMA, p63, calponin, S100 protein and SMMHC. CD117, CEA, GFAP and p16 were variably present. CK20 and HR HPV were negative. STACC needs to be considered in the differential diagnosis of most sinonasal malignancies, particularly poorly differentiated carcinoma, olfactory neuroblastoma and pleomorphic adenoma. Surgery (n = 82), often accompanied by radiation therapy (n = 36), was generally employed. A majority of patients developed a recurrence (n = 52) 2–144 months after initial presentation. Overall mean follow-up was 19.4 years (range 0.4–37.5 years): 46 patients died with disease (mean 6.4 years); 5 were alive with disease (mean 5.4 years), and 35 patients were either alive or had died of unrelated causes (mean 16.3 years). ACC of the SNT is uncommon. Recurrences are common. The following parameters, when present, suggest an increased incidence of either recurrence or dying with disease: mixed site of involvement, high stage disease (stage IV), skull base involvement, tumor recurrence, a solid histology, perineural invasion, bone invasion, and lymphovascular invasion.
doi:10.1007/s12105-013-0487-3
PMCID: PMC3950387  PMID: 24037641
Adenoid cystic carcinoma; Paranasal sinuses; Nasal cavity; Staging; Prognosis; Histology; Immunohistochemistry
8.  Atypical Presentation of Sinonasal Cellular Schwannoma: A Nonsolitary Mass with Osseous, Orbital, and Intracranial Invasion 
Objective Sinonasal cellular schwannoma represents < 4% of head and neck schwannomas. These benign tumors are typically confined to the nasal cavity or ethmoid sinus. We describe an atypical case of sinonasal cellular schwannoma with diffuse paranasal sinus involvement and both intraorbital and intracranial extension.
Results A 62-year-old woman presented with a 6-month history of right orbital proptosis and right-sided headache. Subsequent imaging revealed an invasive paranasal sinus mass extending through the skull base and displacing the right orbit. Preoperative biopsies were not diagnostic but revealed a spindle cell lesion suspicious for malignancy based on lack of encapsulation, infiltration of the sinonasal submucosa, and osseous invasion. The patient underwent open skull base surgery, and pathology confirmed a S100-positive nonencapsulated cellular schwannoma.
Conclusion An atypical case of sinonasal cellular schwannoma with intracranial extension is reported. Its presentation is contrary to the common view that these are isolated solitary lesions of the nasoethmoid region. We suggest that sinonasal cellular schwannoma be considered in the differential diagnosis of a poorly defined invasive paranasal sinus mass, particularly following biopsy.
doi:10.1055/s-0034-1376424
PMCID: PMC4110123  PMID: 25083375
cellular schwannoma; nasal cavity; sinuses; paranasal; intracranial
9.  Evaluation of Sinonasal Diseases by Computed Tomography 
Introduction
Computed Tomography (CT) plays an important diagnostic role in patients with sinonasal diseases and determines the treatment. The CT images clearly show fine structural architecture of bony anatomy thereby determining various anatomical variation, extent of disease and characterization of various inflammatory, benign and malignant sinonasal diseases.
Aim
To evaluate sensitivity and specificity of CT in diagnosis of sinonasal diseases and to characterise the benign and malignant lesions with the help of various CT parameters. Also, to correlate findings of CT with histo-pathological and diagnostic nasal endoscopy/ Functional Endoscopic Sinus Surgery (FESS) findings.
Materials and Methods
In this hospital based prospective study 175 patients with symptomatic sinonasal diseases were evaluated by clinical diagnosis and 16 slice Multi Detector Computed Tomography (MDCT). The details of findings of nasal endoscopy, Functional Endoscopic Sinus Surgery (FESS), histopathological examination and fungal culture were collected in all those cases where those investigations were done. All those findings were correlated with CT findings and statistical analysis was done by using Test statistics (sensitivity, specificity, Positive Predictive Value (PPV), Negative Predictive Value (NPV) and accuracy), Chi-Square test and Z-test for single proportions. Software used in the analysis was SPSS 17.0 version and graph pad prism 6.0 version and p < 0.05 was considered as statistically significant.
Results
CT diagnosis had higher sensitivity, specificity, PPV and NPV in diagnosing various sinonasal diseases in comparison to clinical diagnosis. On correlating CT diagnosis with final diagnosis, congenital conditions have 100% sensitivity and specificity. Chronic sinusitis has 98.3% sensitivity and 97.8% specificity. For fungal sinusitis the sensitivity was 60% and specificity was 99.3%. Polyps have sensitivity of 94.4% and specificity of 98.1%. Benign neoplasms have sensitivity of 90.9% and specificity of 99.2%, malignant neoplasms have sensitivity of 94.1% and specificity of 99.3%. The p-value in all instances was <0.05, i.e. <0.0001 indicating the significance of the findings.
Conclusion
CT is the modality of choice in imaging the sinonasal region for evaluating various congenital, inflammatory, benign and malignant pathologies and associated complications thereby planning the further management of the patient. CT is the best modality of choice for evaluating the bone erosion or destruction. The potential pitfalls to differentiate on CT are fungal sinusitis and dense secretions.
doi:10.7860/JCDR/2016/23197.8826
PMCID: PMC5198426  PMID: 28050473
Diagnostic nasal endoscopy; Functional endoscopic sinus surgery; Sinonasal pathologies
10.  INI1 (SMARCB1)-Deficient Sinonasal Carcinoma: A Clinicopathologic Report of 2 Cases 
Head and Neck Pathology  2016;11(2):256-261.
Poorly differentiated sinonasal malignancies are amongst the hardest differential diagnoses in pathology, owing to the large number of rare entities that arise there. Complicating the matter is that most pathologists, including those with experience in head and neck pathology, have little experience in any one of these rare entities. Most patients with sinonasal carcinoma present with locally advanced disease and in the past a combination of chemotherapy, radiotherapy, and surgery would usually be recommended without the specific disease subtype playing a large part of the decision making. However, in the era of “precision medicine” and targeted therapies, the specific tumour subtype and an accurate diagnosis will become increasingly important even for the so-called “undifferentiated carcinoma”. Specific entities that tend to enter into the differential diagnosis include olfactory neuroblastoma, sinonasal undifferentiated carcinoma (SNUC), and non-keratinizing squamous cell carcinoma (viral and non-viral). However, recent new entities, such as NUT-midline carcinoma also have to be considered. Recently it was found that a subset of tumours originally diagnosed as one of the aforementioned entities all demonstrated loss of the ubiquitously expressed protein Integrase Interactor 1 (INI1; SMARCB1). These tumours were often basaloid with at least partial rhabdoid differentiation and most were considered a part of the SNUC spectrum. In this report, we describe two additional cases of INI1-deficient sinonasal carcinoma prospectively identified, both of which appeared to have a marked response to neo-adjuvant chemoradiation, a finding not previously described.
doi:10.1007/s12105-016-0752-3
PMCID: PMC5429275  PMID: 27644951
SNUC; INI1; SMARCB-1; Sinonasal; Carcinoma
11.  Magnetic Resonance Imaging Versus Computed Tomography and Different Imaging Modalities in Evaluation of Sinonasal Neoplasms Diagnosed by Histopathology 
Objective
The study purpose was to detect the value of magnetic resonance imaging (MRI) compared to computed tomography (CT) and different imaging modalities as conventional radiology in evaluation of sinonasal neoplasms diagnosed by Histopathology.
Methods
Thirty patients (16 males and 14 females) were complaining of symptoms related to sinonasal tract. After thorough clinical and local examination, the patients were subjected to the following: conventional radiography, CT, MRI, and histopathological examination.
Results
The nasal cavity was the most commonly involved site with sinonasal malignancies followed by the maxillary sinuses. The least commonly affected site was the frontal sinuses. Benign sinonasal tumors were present in 14 cases. The most common benign lesion was juvenile nasopharyngeal angiofibroma (6 cases), followed by inverted papilloma (3 cases). While malignant sinonasal tumors were present in 16 cases, squamous cell carcinoma was present in 5 cases, and undifferentiated carcinoma, in 3 cases. Lymphoepithelioma and non-Hodgkin lymphomas were present in 2 cases each, while adenocarcinoma, chondrosarcoma, adenoid cystic carcinoma, and rhabdomyosarcoma were present in 1 case each.
Conclusion
MRI with its superior soft tissue contrast and multiplanar capability is superior to CT in pretreatment evaluation of primary malignant tumors of sinonasal cavity.
doi:10.4137/CMENT.S10678
PMCID: PMC3791956  PMID: 24179408
magnetic resonance imaging; computed tomography; sinonasal tumor
12.  Sinonasal malignancies: a 10-year review in a tertiary health institution. 
Sinonasal malignancy is a cause of otorhinolaryngologic morbidity and mortality in West Africa. However, there is a dearth of information in the literature on its clinicopathologic presentation in West Africa. It is our aim to determine the prevalence of sinonasal malignancy and highlight the clinicopathologic features in our environment. A 10-year retrospective review of cases with histologically diagnosed malignant sinonasal tumors in University College Hospital, Ibadan, Oyo State, Nigeria was carried out. There were 82 cases-56 (68.29%) males and 26 (31.71%) females-whose ages ranged from 4-69 years. Epistaxis, rhinorrhea and nasal blockage were seen in all patients; other symptoms were facial [76 (93%)], oral cavity [48 (59%)], ophthalmic [33 (40%)] and [otologic 21 (25%)]. Squamous cell carcinoma accounted for 69/75 (92%) of epithelial tumors, and malignant lymphoma accounted for 4/7 (57%) of nonepithelial tumors. Advanced disease was the predominant presentation in our series, stage 3 in 59 (79%) and stage 4 in 12 (16%) cases. Therefore, health education on early presentation to hospitals and efforts at early detection of the disease are needed in order to achieve cure. Industrial workers should always wear face masks to protect their nasal cavity.
Images
PMCID: PMC2575924  PMID: 18229778
13.  Carcinoma Ex-Schneiderian Papilloma (Malignant Transformation): A Clinicopathologic and Immunophenotypic Study of 20 Cases Combined with a Comprehensive Review of the Literature 
Head and Neck Pathology  2014;8(3):269-286.
Schneiderian papilloma (SP) are uncommon tumors with malignant transformation even less common. The histologic criteria to define malignant transformation are not well developed nor is the immunohistochemical profile reported in a large series of carcinomas. 20 cases of malignant transformation of SP included 7 females and 13 males, aged 38–86 years (mean 60.7 years). Patients presented most frequently with a mass (n = 11) and obstructive symptoms (n = 7), present for 38.7 months (mean). Most patients had no previous history of SP (n = 13); metachronous carcinoma was identified in 7 patients an average of 34.4 months after the first diagnosis of SP, with 1–4 recurrences of SP. With a mean size of 4.1 cm, the majority of tumors involved a combination of more than one anatomic site (n = 10), followed by the maxillary sinus only (n = 5) or nasal cavity only (n = 3). Histologically, 17 were inverted and 3 exophytic type SP. There were 17 squamous cell carcinomas, 2 mucoepidermoid carcinomas and 1 sinonasal undifferentiated carcinoma, comprising from 10 to 95 % of the tumor volume. Malignant histologic features included atypical mitoses, necrosis, bone invasion, lymphovascular invasion, decreased transmigrating neutrophils, paradoxical maturation, dyskeratosis and/or perineural invasion (n = 3). Patients tended to present with advanced stage (n = 14, Stage III and IV). Immunohistochemical studies showed positive reactions in the malignancies for CK5/6 (86 %), p63 (86 %), CK7 (luminal, 50 %), p53 (83 %), and p16 (25 %). In situ hybridization detected human papillomavirus in 26 %. Surgery was often accompanied by radiation therapy (n = 13), with a mean of 2.4 years of follow-up. Five patients developed a recurrence between 0.8 and 3.3 years. Carcinomas ex-SP are less common and are associated with better outcome than previously reported. Patients tend to present with a synchronous carcinoma, developing in an inverted type SP, with squamous cell carcinoma the most common malignancy. Development of metachronous carcinomas ex-SP was always preceded by SP recurrence in this series.
doi:10.1007/s12105-014-0527-7
PMCID: PMC4126921  PMID: 24519376
Sinonasal tract; Schneiderian papilloma; Malignant transformation; Review; Immunohistochemistry; HPV
14.  Sinonasal Tract Mucoepidermoid Carcinoma: A Clinicopathologic and Immunophenotypic Study of 19 Cases Combined with a Comprehensive Review of the Literature 
Head and Neck Pathology  2011;6(2):191-207.
Primary sinonasal tract mucoepidermoid carcinomas (MEC) are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. The design of this study is retrospective. Nineteen cases of MEC included 10 females and 9 males, aged 15–75 years (mean, 52.7 years); males, on average were younger by a decade than females (47.2 vs. 57.7 years). Patients presented most frequently with a mass, obstructive symptoms, pain, and/or epistaxis present for a mean of 12.6 months. The majority of tumors involved the nasal cavity alone (n = 10), maxillary sinus alone (n = 6), or a combination of the nasal cavity and paranasal sinuses (n = 3) with a mean size of 2.4 cm. Most patients presented at a low clinical stage (n = 15, Stage I & II), with only 4 patients presenting with Stage III disease. Histologically, the tumors were often invasive (bone or perineural invasion), with invasion into minor mucoserous glands. Surface involvement was common. The neoplastic cells were composed of a combination of squamoid cells, intermediate cells, and mucocytes. Cystic spaces were occasionally large, but the majoritywere focal to small. Pleomorphism was generally low grade. Necrosis (n = 5) and atypical mitotic figures (n = 6) were seen infrequently. Over half of the tumors were classified as low grade (n = 11), with intermediate (n = 4) and high grade (n = 4) comprising the remainder. Mucicarmine was positive in all cases tested. Immunohistochemical studies showed positive reactions for keratin, CK5/6, p63, CK7, EMA, and CEA in all cases tested, while bcl-2 and CD117 were rarely positive. GFAP, MSA, TTF-1, and S100 protein were non-reactive. p53 and Ki-67 were reactive to a variable degree. MEC need to be considered in the differential diagnosis of a number of sinonasal lesions, particularly adenocarcinoma and necrotizing sialometaplasia. The patients were separated into stage I (n = 9), stage II (n = 6), and stage III (n = 4), without any patients in stage IV at presentation. Surgery occasionally accompanied by radiation therapy (n = 2) was generally employed. Six patients developed a recurrence, with 5 patients dying with disease (mean, 2.4 years), while 14 patients are either alive (n = 9) or had died (n = 5) of unrelated causes (mean, 14.6 years). MEC probably arises from the minor mucoserous glands of the upper aerodigestive tract, usually presenting in patients in middle age with a mass. Most patients present with low stage disease (stage I and II), although invasive growth is common. Recurrences develop in about a third of patients, who experience a shorter survival (mean, 6.5 years). The following parameters, when present, suggest an increased incidence of recurrence or dying with disease: size ≥4.0 cm (P = 0.034), high mitotic count (P = 0.041), atypical mitoses (P = 0.007), mixed anatomic site (P = 0.032), development of recurrence (P = 0.041), high tumor grade (P = 0.007), and higher stage disease (P = 0.027).
doi:10.1007/s12105-011-0320-9
PMCID: PMC3370018  PMID: 22183767
Sinonasal tract; Mucoepidermoid carcinoma; Nasal cavity; Maxillary sinus, ethmoid sinus; Frontal sinus; Review; Meta-analysis; Immunohistochemistry; Prognosis; Outcome; Staging; Differential diagnosis; Carcinoma
15.  Clinico-pathological profile of sinonasal masses: An experience in tertiary care hospital of Uttarakhand 
Background:
The purpose of this study was to classify various types of non-neoplastic and neoplastic lesions presenting as sinonasal mass and characterize their clinico-pathological profile in a tertiary care center in the state of Uttarakhand.
Materials and Methods:
This was a prospective study where 110 cases of sinonasal masses were included over a period of 12 months. Clinico-pathological study was carried out in these cases. A provisional diagnosis was made after clinical assessment and radiologic investigations, but final diagnosis was made after histopathologic examination.
Observations:
The number of non-neoplastic lesions were more than the neoplastic lesion, 60% versus 40% respectively. In the neoplastic group, 19.8% and 23.76% patients presented with benign and malignant lesion, respectively. The incidence was more predominant in the age group of 11-20 years (22.72%) with male to female ratio of 1.08:1. In our study, among non-neoplastic lesions the occurrence of sinonasal polyps was highest seen in 80.30% cases. In neoplastic lesions, angiofibroma was most common benign lesion seen in 35% cases. Carcinoma nasal cavity was the commonest malignant lesion seen in 45.83% cases. In 3.63% patients, clinical and radiologic diagnosis was not correlated with histopathologic diagnosis. Only two cases required immuno-histocytochemistry to confirm the final diagnosis.
Conclusion:
We concluded that for proper evaluation of a sinonasal mass, clinical, radiologic, and histopathologic evaluation should be carried out conjointly in all the cases. Histopathology always gives a confirmatory diagnosis but in few cases immuno-histocytochemistry becomes the ultimate diagnostic technique for correct and timely intervention.
doi:10.4103/0975-5950.111375
PMCID: PMC3700153  PMID: 23833494
Histopathology; neoplastic lesion; sinonasal mass
16.  Sinonasal involvement in systemic vasculitides and cocaine-induced midline destructive lesions: Diagnostic controversies 
Allergy & Rhinology  2013;4(2):e94-e99.
Multiple systemic diseases produce various clinical manifestations in the sinonasal area. They usually appear as difficult-to-diagnose disease processes with slow, atypical clinical courses. The aim of this study was to evaluate the sinonasal manifestations of systemic vasculitides, highlighting key points for diagnosis and differential diagnosis with other pathological entities, especially cocaine-induced midline destructive lesions (CIMDL). A retrospective study was performed of 10 patients treated in our hospital during the last 5 years with an initial diagnosis of systemic vasculitides with sinonasal involvement: eight patients with granulomatosis with polyangiitis (GPA; new nomenclature for Wegener granulomatosis) and two patients with Churg-Strauss syndrome (CSS). The study variables were clinical presentation, nasal endoscopy results, maxillofacial scan results, nasal biopsy results, erythrocyte sedimentation rate, and autoimmune antibody levels. The definitive diagnosis was GPA in six (60%) patients, CSS in two (20%) patients, and CIMDL in two (20%) patients. Nasal symptoms were similar in all patients, but nasal polyps were present in only one patient with CSS. Systemic manifestations were absent in patients with CIMDL. Likewise, peripheral eosinophilia was observed only in the two patients with CSS. Specific positive biopsy specimens were obtained in six patients (all six patients with GPA, one with CSS, and one with CIMDL). Antineutrophil cytoplasmic antibodies (ANCA) were positive in all patients with GPA (proteinase 3 antigen in five patients and myeloperoxidase in one patient), and perinuclear ANCA was positive in one patient with CIMDL; however, this patient showed an undefined pattern. Finally, the response to treatment was adequate in all patients excluding those with CIMDL. GPA and CIMDL syndromes pose a difficult differential diagnosis because they have common clinical, serological, and histological presentations. Negative histological results do not exclude the diagnosis of sinonasal vasculitides. The absence of systemic manifestations and the lack of response to treatment will lead to the confirmation of CIMDL syndrome in a cocaine user. Otolaryngologists play an important role in the early and differential diagnosis of these diseases.
doi:10.2500/ar.2013.4.0051
PMCID: PMC3793120  PMID: 24124643
ANCA; autoimmune antibodies; Churg-Strauss syndrome; cocaine user; eosinophilia; differential diagnosis; histological diagnosis; nasal polyps; systemic diseases; Wegener's granulomatosis
17.  Human Papillomavirus-Related Carcinomas of the Sinonasal Tract 
High risk human papillomavirus (HPV) is an established cause of head and neck carcinomas arising in the oropharynx. The presence of HPV has also been reported in some carcinomas arising in sinonasal tract, but little is known about their overall incidence or their clinicopathologic profile. The surgical pathology archives of The Johns Hopkins Hospital were searched for all carcinomas arising in the sinonasal tract from 1995 to 2011, and tissue microarrays were constructed. P16 immunohistochemistry and DNA in situ hybridization for high-risk types of HPV were performed. Demographic and clinical outcomes data were extracted from patient medical records. Of 161 sinonasal carcinomas, 34 (21%) were positive for high risk HPV DNA, including type 16 (82%), type 31/33 (12%), and type 18 (6%). HPV-positive carcinomas consisted of 28 squamous cell carcinomas and variants (15 non- or partially-keratinizing, 4 papillary, 5 adenosquamous, 4 basaloid), 1 small cell carcinoma, 1 sinonasal undifferentiated carcinoma, and 4 carcinomas that were difficult to classify but exhibited adenoid cystic carcinoma-like features. Immunohistochemistry for p16 was positive in 59/161 (37%) cases, and p16 expression strongly correlated with the presence of HPV DNA: 33 of 34 (97%) HPV positive tumors exhibited high p16 expression, whereas only 26 of 127 (20%) HPV negative tumors were p16 positive (p < .0001). The HPV-related carcinomas occurred in 19 men and 15 women ranging in age from 33 to 87 years (mean 54). A trend toward improved survival was observed in the HPV-positive group (hazard ratio=0.58, 95% confidence interval [0.26, 1.28]). The presence of high risk HPV in 21% of sinonasal carcinomas confirms HPV as an important oncologic agent of carcinomas arising in the sinonasal tract. While non-keratinizing squamous cell carcinoma is the most common histologic type, there is a wide morphologic spectrum of HPV-related disease that includes a variant that resembles adenoid cystic carcinoma. The distinctiveness of these HPV-related carcinomas of the sinonasal tract with respect to risk factors, clinical behavior, and response to therapy remains to be clarified.
doi:10.1097/PAS.0b013e3182698673
PMCID: PMC3545097  PMID: 23095507
Human papillomavirus; sinonasal carcinoma; squamous cell carcinoma; adenoid cystic carcinoma; sinonasal undifferentiated carcinoma
18.  Ewing’s Family of Tumors of the Sinonasal Tract and Maxillary Bone 
Head and Neck Pathology  2010;5(1):8-16.
The Ewing’s family of tumors (EFT) are malignant neoplasms affecting children and young adults. Most cases arise in the long bones or the pelvis. Primary EFT of head and neck is uncommon and primary sinonasal EFT is even rarer. Previous studies have not focused on the sinonasal region specifically, and the published literature on sinonasal EFT consists of sporadic case reports. Fourteen cases of sinonasal EFT were available and had H&Es for review and immunohistochemical stains for CD99, S100, keratins, synaptophysin and desmin. FISH or RT-PCR was performed for EWSR1 abnormalities on 8 cases. The 14 identified patients included 5 males and 9 females, ranging from 7–70 years of age (mean 32.4 years). Tumors involved nasal cavity (5), sinuses (5) or both (4). Five patients had dural, orbital or brain involvement. The majority involved bone radiologically and/or microscopically. All cases were composed of small cells with variable cytoplasmic clearing. Focal or prominent nesting was noted in most cases. All cases were positive for CD99. Keratins (AE1/3 and/or CAM5.2), S100 and synaptophysin were positive in 4, 3 and 5 cases, respectively. All cases were negative for desmin. The 8 cases tested by FISH or RT-PCR were positive for EWSR1 abnormalities. Follow-up in 8 patients ranged from 1–168 months (average 11.3 m) showing 1 death due to metastatic disease, 1 death due to local disease, 1 patient alive with metastases and 5 patients disease-free at last follow-up. Interestingly, however, an analysis of the literature suggests a better prognosis for sinonasal EFT than EFT overall.
doi:10.1007/s12105-010-0227-x
PMCID: PMC3037459  PMID: 21107767
Ewing’s family of tumors; Sinonasal; Maxillary bone; Olfactory neuroblastoma
19.  Cavernous sinus involvement is not a risk factor for the primary tumor site treatment outcome of Sinonasal adenoid cystic carcinoma 
Background
Sinonasal adenoid cystic carcinoma is a rare malignancy of the head and neck. Cavernous sinus invasion from sinonasal adenoid cystic carcinoma and its related management have rarely been investigated. This study evaluated the relationship between treatment outcome and cavernous sinus involvement in addition to other parameters.
Methods
A retrospective case series study was conducted at a tertiary referral center. The medical records of 47 patients diagnosed with primary sinonasal adenoid cystic carcinoma between 1984 and 2015 were retrospectively reviewed. The survival impact of the primary treatment modalities and the anatomic sites of tumor involvement were analyzed.
Results
Cavernous sinus invasion was observed in 8 patients (17%), of whom 7 had ACC tumors originating from the maxillary sinus. The results of univariate analysis revealed that tumor stage, primary surgery, and the absence of skull-base and infratemporal fossa invasion were associated with better overall survival (P = 0.033, P = 0.012, P = 0.011, and P = 0.040, respectively) and better disease-free survival (P = 0.019, P = 0.001, P = 0.017, and P = 0.029, respectively). Multivariate analysis identified primary surgery as the only independent prognostic factor for disease-free survival (P = 0.026). Cavernous sinus invasion by sinonasal adenoid cystic carcinoma was not associated with worse overall survival or disease-free survival (P = 0.200 and P = 0.198, respectively).
Conclusions
Because maxillary adenoid cystic carcinoma is associated with a higher rate of cavernous sinus invasion, such cases warrant caution during preoperative planning. Primary surgery as the initial therapy provides better locoregional control and survival for patients with sinonasal adenoid cystic carcinoma. Cavernous sinus invasion did not significantly impact survival; thus, it should not be regarded as a contraindication for curative treatment.
doi:10.1186/s40463-018-0257-z
PMCID: PMC5800036  PMID: 29402308
Sinonasal cancer; Adenoid cystic carcinoma; Cavernous sinus; Head and neck; Surgery; Chemoradiation
20.  Spectrum of Sinonasal Tumors: A 10-year Experience at a Tertiary Care Hospital in North India 
Oman Medical Journal  2015;30(6):435-440.
Objective
Sinonasal tumors are a highly heterogeneous group of tumors that account for less than 1% of all cancers. Precise diagnosis is essential because the natural history, treatment, and prognosis vary for different neoplasms. We reviewed 610 cases of sinonasal lesions to understand this entity better.
Methods
A total of 610 sinonasal biopsies/specimens were received over a period of 10 years (2004–14). All the samples were processed and stained with hematoxylin and eosin. Special staining for microorganisms and immunohistochemistry were performed where indicated.
Results
Benign lesions were 72% with nasal polyp being the most common. Malignant lesions made up 21.2% of lesions with nasopharyngeal carcinoma being the most common while 7.7% cases were inflammatory/non-neoplastic. The most common cause for this was fungal sinusitis. The maxillary sinus was the most frequently involved site in all the lesions (62%). The average age of presentation was 40 years old, and the male to female ratio was 3:1 in those with benign tumors. The average age of presentation in the malignant and inflammatory group were 45 and 43 years, respectively, with an almost equal sex ratio in both categories. Computed tomography and magnetic resonance imaging scans were done in virtually all cases to assess the extent of the tumor as well as bony destruction. Local recurrence was the most frequent cause of treatment failure.
Conclusion
Tumors of the nasal cavity are often grouped with those in the paranasal sinuses. Benign tumors constituted approximately 75% of tumors in our hospital. Squamous cell carcinoma was the most common malignancy of this region. These tumors need to be studied closely and accurately because their proximity to vital structures pose significant challenges for their treatment and may be the source of significant patient morbidity.
doi:10.5001/omj.2015.86
PMCID: PMC4678446  PMID: 26674709
Carcinoma, Squamous Cell; Paranasal Sinuses; Nasal Polyps; Histology
21.  Sinonasal Tract Angiosarcoma: A Clinicopathologic and Immunophenotypic Study of 10 Cases with a Review of the Literature 
Head and Neck Pathology  2007;1(1):1-12.
Background    Primary sinonasal tract angiosarcoma are rare tumors that are frequently misclassified, resulting in inappropriate clinical management. There are only a few reported cases in the English literature. Materials and Methods    Ten patients with sinonasal tract angiosarcoma were retrospectively retrieved from the Otorhinolaryngic Registry of the Armed Forces Institute of Pathology. Results    Six males and four females, aged 13 to 81 years (mean, 46.7 years), presented with epistaxis and bloody discharge. Females were on average younger than their male counterparts (37.8 vs. 52.7 years, respectively). The tumors involved the nasal cavity alone (n = 8) or the maxillary sinus (n = 2), with a mean size of 4.3 cm; the average size was different between the genders: males: 2.8 cm; females: 6.4 cm. Histologically, all tumors had anastomosing vascular channels lined by remarkably atypical endothelial cells protruding into the lumen, neolumen formation, frequent atypical mitotic figures, necrosis, and hemorrhage. All cases tested (n = 6) demonstrated immunoreactivity with antibodies to Factor VIII-RA, CD34, CD31, and smooth muscle actin, while non-reactive with keratin and S-100 protein. The principle differential diagnosis includes granulation tissue, lobular capillary hemangioma (pyogenic granuloma), and Kaposi’s sarcoma. All patients had surgery followed by post-operative radiation (n = 4 patients). Follow-up was available in all patients: Six patients died with disease (mean, 28.8 months); two patients had died without evidence of disease (mean, 267 months); and two are alive with no evidence of disease at last follow-up (mean, 254 months). Conclusions    Sinonasal tract angiosarcoma is a rare tumor, frequently presenting in middle-aged patients as a large mass usually involving the nasal cavity with characteristic histomorphologic and immunophenotypic features. Sinonasal tract angiosarcoma will often have a poor prognosis making appropriate separation from other conditions important.
doi:10.1007/s12105-007-0017-2
PMCID: PMC2807511  PMID: 20614274
Angiosarcoma; Sinonasal tract; Nasal cavity; Vascular; Hemangioma; Sarcoma; Immunohistochemistry; Prognosis; Survival; Differential diagnosis
22.  Management of Sinonasal Tumors: Prognostic Factors and Outcomes: A 10 Year Experience at a Tertiary Care Hospital 
Sinonasal malignancies are said to be a highly heterogeneous group of cancers, accounting for less than 1 % of all cancers and less than 3 % of all upper aerodigestive tract tumors. Originating from any histologic components of the sinonasal cavity, the histopathology of these tumors is diverse. Accordingly, treatment options vary, surgery being the mainstay in most of them. Recurrence rates differs with each histological type of tumor, dependent on various factors. In this article, we have tried to identify the prevalent characteristics of sinonasal malignancies and to outline the prognostic factors affecting the outcome. It is a retrospective study design with a total number of 102 patients. Patients diagnosed with sinonasal malignancies were included and any patient previously operated outside our institute or having received prior radiation or chemotherapy were excluded. The patients were selected over a period of 10 years, from 2000 to 2010. Data was analyzed using SPSS 17. Majority of the sinonasal tumors were squamous cell carcinomas involving the maxillary sinus. Locoregional recurrence was found to be more frequent in patients with positive neck nodes on final histopathology. Sinonasal malignancies are mostly squamous cell in variety and recurrence of these rare entities is dependent on the histological variety and the presence of positive neck nodes.
doi:10.1007/s12070-013-0650-x
PMCID: PMC3718929  PMID: 24427634
Sinonasal malignancy; Locoregional recurrence; Squamous cell carcinoma; Positive neck nodes
23.  Imaging Characteristics of Malignant Sinonasal Tumors 
Malignancies of the nasal cavity and paranasal sinuses account for 1% of all malignancies and 3% of malignancies of the upper aerodigestive tract. In the sinonasal tract, nearly half of all malignancies arise in the nasal cavity, whereas most of the remaining malignancies arise in the maxillary or ethmoid sinus. Squamous cell carcinoma is the most common histological subtype of malignant tumors occurring in this area, followed by other epithelial carcinomas, lymphomas, and malignant soft tissue tumors. Although many of these tumors present with nonspecific symptoms, each tumor exhibits characteristic imaging features. Although complex anatomy and various normal variants of the sinonasal tract cause difficulty in identifying the origin and extension of large sinonasal tumors, the invasion of vital structures such as the brain, optic nerves, and internal carotid artery affects patients’ prognosis. Thus, diagnostic imaging plays a key role in predicting the histological subtype and in evaluating a tumor extension into adjacent structures. This article describes the computed tomography and magnetic resonance imaging findings for malignant sinonasal tumors.
doi:10.3390/jcm6120116
PMCID: PMC5742805  PMID: 29211048
sinonasal tract; malignant tumor; CT; MRI
24.  Malignant transformation of sinonasal inverted papilloma: A retrospective analysis of 32 cases 
Oncology Letters  2014;8(6):2637-2641.
Sinonasal inverted papillomas (SNIPs) are derived from the benign tumors of the epithelial cells and have the potential to recur and exhibit malignant characteristics. The aim of the present study was to investigate the clinicopathological features and prognosis of patients with malignant transformation of SNIP. A total of 32 consecutive cases, who were patients at the Department of Otorhinolaryngology Head and Neck Surgery, Tianjin Huanhu Hospital from January 1991 to January 2008, were retrospectively reviewed. Survival rates and prognostic factors were calculated using the Kaplan-Meier method and multivariate Cox model survival analysis. The malignancy accounted for 8.99% of all types of SNIP. There were 25 males and seven females, and the median age of onset was 56.5 years. The sites of tumor included 22 in the nasal cavity and ethmoid sinuses, and 10 in the maxillary sinus. The tumors included 21 high-grade tumors, eight intermediate-grade tumors and three low-grade tumors. The number of patients with T1, T2, T3 and T4 stage disease was three, 10, 16 and three, respectively, according to the American Joint Committee on Cancer staging method. Based on the percentage of malignant cells in the entire tumor tissue, five patients had grade I tumors, five had grade II, eight had grade III and14 had grade IV. Among the 32 patients, three cases exhibited distant metastasis, and 19 patients underwent surgery plus postoperative radiotherapy, 10 underwent surgery alone and three underwent radiotherapy alone. The 5-year survival rate was 72.5% and the median overall survival time was 62.2 months. Kaplan-Meier univariate survival analysis indicated that the clinical stage and treatment method were prognostic factors, and multivariate Cox model survival analysis confirmed that the clinical stage and treatment method were independent factors for overall survival (relative risk: 4.211 and 0.312, respectively; P<0.05 for both). T3 and T4 staging and mono-treatment were associated with poor patient survival. Overall, the present study identified that the morbidity of SNIP-associated malignancy was low, the clinicopathological features were not specific, and the prognosis was improved compared with other types of sinonasal squamous cell carcinoma. The clinical stage and treatment method were found to affect the prognosis, and surgery plus postoperative radiotherapy was the predominant form of treatment. The present study may improve the understanding of the prognosis for patients with malignant SNIP in the future.
doi:10.3892/ol.2014.2539
PMCID: PMC4214400  PMID: 25360173
sinonasal tumor; inverted papilloma; malignancy; squamous cell carcinoma
25.  Schneiderian papillomas: Comparative review of exophytic, oncocytic, and inverted types 
Background:
Sinonasal papillomas are benign epithelial neoplasms arising from Schneiderian mucosa. The three subtypes, exophytic, oncocytic, and inverted (inverted papilloma [IP]), should be distinguished from one another histopathologically. This study (1) highlights the histopathological and clinical differences between the Schneiderian papilloma subtypes and (2) identifies clinical features that potentially predict papilloma subtypes.
Methods:
A retrospective review was performed of patients with Schneiderian papillomas over an 11-year period.
Results:
Seventy patients with sinonasal papillomas who underwent sinus surgery were identified. There were 50 (71%) male and 20 (29%) female subjects diagnosed at an average age of 53 years (range, 13–80 years). Exophytic (n = 25), oncocytic (n = 9), and IP (n = 37) were identified. IP was associated with transformation into squamous cell carcinoma in three (8%) cases and dysplasia in three (8%) cases. Neither oncocytic nor exophytic subtypes were associated with dysplasia or malignancy. On multivariate analysis of potential predictors of papilloma subtype, history of chronic rhinosinusitis (CRS) and location of papilloma were significantly associated with papilloma subtype. Using classification and regression tree model, papilloma subtypes can be predicted based on presence or absence of CRS and papilloma location with nominal 82.4% accuracy.
Conclusion:
The inverted and exophytic type are the most common sinonasal papillomas, with the inverted type having an 8% rate of malignant transformation in this study. In contrast, the oncocytic type was not associated with dysplasia or malignancy in our series despite reports in the literature indicating malignant potential. History of CRS and papilloma location can provide clues to the histological subtype, which is important for surgical planning and patient counseling.
doi:10.2500/ajra.2013.27.3904
PMCID: PMC3901443  PMID: 23883810
Cylindrical cell; exophytic; inverted; oncocytic; Schneiderian papillomas; sinonasal papillomas

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