Chronic invasive fungal rhinosinusitis vs sinonasal squamous cell carcinoma: the differentiating value of MRI.

OBJECTIVES: To investigate MRI features in discriminating chronic invasive fungal rhinosinusitis (CIFRS) from sinonasal squamous cell carcinomas (SNSCC). METHODS: MRI findings of 33 patients with CIFRS and 47 patients with SNSCC were retrospectively reviewed and compared. Multivariate logistic regression analysis was performed to identify significant imaging features in distinguishing between CIFRS and SNSCC. The ROC curves and the AUC were used to evaluate diagnostic performance. RESULTS: There were significant differences in cavernous sinus involvement (p < 0.001), sphenoid sinus involvement (p < 0.001), meningeal involvement (p = 0.024), T2 signal intensity (p = 0.006), and enhancement pattern (p < 0.001) between CIFRS and SNSCC. Multivariate logistic regression analysis identified cavernous sinus involvement (odds ratio [OR] = 0.06, 95% confidence interval [95% CI] = 0.02-0.20) and sphenoid sinus involvement (OR = 0.14, 95% CI = 0.05-0.45) as significant indicators for CIFRS and T2 isointensity to gray matter (OR = 4.44, 95% CI = 1.22-16.22) was a significant indicator for SNSCC. ROC curve analysis showed the AUC from a combination of three imaging features was 0.95 in differentiating CIFRS and SNSCC. CONCLUSIONS: MRI showed significant differences between CIFRS and SNSCC features. In immunocompromised patients, a sinonasal hypointense mass on T2WI with septal enhancement or loss of contrast enhancement, and involvement of cavernous sinus, sphenoid sinus, and meninges strongly suggest CIFRS. KEY POINTS: • Chronic invasive fungal rhinosinusitis (CIFRS) is often difficult to distinguish from sinonasal squamous cell carcinomas (SNSCC) in clinical practice. • Cavernous sinus and sphenoid sinus involvement appear to be significant indicators for CIFRS. T2 isointensity to gray matter appears to be a significant indicator for SNSCC. • Loss of contrast enhancement and septal enhancement can be used to distinguish CIFRS from SNSCC with a high degree of specificity.

Varied presentation of sinonasal phosphaturic mesenchymal tumour: report of a case series with follow-up.

PURPOSE: Phosphaturic mesenchymal tumour (PMT) of the paranasal sinuses is a rare tumour that is associated with oncogenous osteomalacia causing predominant musculoskeletal symptoms. We present a series of eight patients diagnosed to have PMT of the paranasal sinuses with varied presentation and highlight the appropriate evaluation required to arrive at the diagnosis. METHODS: Retrospective review of eight patients diagnosed to have PMT-causing tumour-induced osteomalacia with follow-up data. RESULTS: Eight patients, 4 males and 4 females, aged 36-67 years (mean = 44 years) presented with vague musculoskeletal symptoms (6 patients) or epistaxis (3 patients). Six patients were found to have hypophosphatemia, phosphaturia and raised FGF-23 levels preoperatively. All eight patients were found to have a tumour in the nose and/ paranasal sinuses with one patient having intracranial extension. All patients were treated with endoscopic excision of these tumours which resulted in resolution of symptoms and normalisation of biochemical abnormalities. In addition, one patient required a craniofacial resection. Histopathological features were consistent with PMT mixed connective tissue variant. Two atypical patients were seen. The longest follow-up was for 5 years and there was no evidence of recurrence during the follow-up period in any patient. CONCLUSION: Diagnosis of PMT of the paranasal sinuses causing oncogenous osteomalacia requires a high index of suspicion when there are no ENT symptoms. Appropriate biochemical tests and histopathology lead to the correct diagnosis. Total endoscopic surgical excision leads to a good outcome.

Low-Grade Papillary Schneiderian Carcinoma of the Sinonasal Cavity and Temporal Bone.

OBJECTIVES:: The aim of this study was to further characterize a newly described neoplasm, low-grade papillary Schneiderian carcinoma, occurring simultaneously in the sinonasal cavity and mastoid. Additionally, the authors review the only 2 similar cases within the literature and describe the common clinical features, radiographic findings, and pathologic characteristics of this exceptionally rare disease process. METHODS:: Chart review for single patient, review of literature. RESULTS:: The patient presented with bilateral nasal obstruction. Computed tomography revealed a left sinonasal mass with skull base hyperostosis, and follow-up magnetic resonance imaging showed a concomitant olfactory groove meningioma. Examination showed a bilateral, completely obstructing sinonasal mass with skip areas, and biopsy confirmed inverted papilloma (human papilloma virus strains 16 and 18 indeterminate). The patient underwent bilateral endoscopic sinus surgery, left medial maxillectomy, and left partial nasopharyngectomy. Given her multifocal disease, she was advised that she would require additional excision, but was lost to follow up. One year later she developed acute left facial paralysis. Magnetic resonance imaging demonstrated an enhancing mass in the left mastoid with enhancement along the Eustachian tube in addition to her known recurrent sinonasal disease. Simultaneous endoscopic sinus surgery and mastoidectomy were performed. Polypoid tissue was removed from the nasopharynx, mesotympanum, epitympanum, and retrofacial air cells. Immunohistochemistry showed that cells stained positive for p63 and dermCK and negative for synaptophysin. Morphologically, cells were bland, without classic stromal invasion, retaining their smooth, cystic, and papillary features, despite their increased depth within the tissue. Upon further review and consultation with an outside pathologist, a diagnosis of low-grade papillary Schneiderian carcinoma was made. The patient was referred for radiation therapy and is disease free at 3-month follow-up, with return of her facial function. CONCLUSIONS:: This case represents the first report of concurrent low-grade papillary Schneiderian carcinoma of both the nasal cavity and mastoid. It emphasizes the importance of recognizing this new entity through pathologic analysis and suspecting it when the clinical course does not follow an expected pattern.

Intralesional Bevacizumab for the Treatment of Recurrent Sinonasal Hemangioma.

INTRODUCTION:: Sinonasal hemangioma is a rare benign tumor of vascular endothelial cells. The pathogenesis is closely linked to abnormalities in the vascular endothelial growth factor signaling pathway. Multiple treatment modalities are available, though wide local excision remains the preferred treatment. Bevacizumab, an anti-vascular endothelial growth factor monoclonal antibody, has known efficacy against hemangiomas, though there are no cases of sinonasal hemangiomas managed with bevacizumab. METHODS:: Case report. RESULTS:: The authors review the case of a 67-year-old man with a left-sided nasal hemangioma originating from the nasal septum. He presented with progressive left nasal obstruction and recurrent epistaxis. At the time of his presentation, the lesion had recurred after 1 excision/polypectomy at an outside institution. He then underwent revision surgery via wide local excision and septoplasty. After tumor recurrence following the revision surgery, the hemangioma was noted to recur on 1 year postoperative surveillance nasal endoscopy. In-office intralesional injection of 50 mg bevacizumab was then performed under endoscopic visualization. No improvement in the tumor size was noted at 2 months after injection, with the tumor measuring 1.5 cm. At the 10-month surveillance clinical visit following injection, the tumor had dramatically involuted to 3 mm in greatest dimension. The patient reported complete resolution of his primary symptoms of epistaxis and nasal obstruction. CONCLUSIONS:: This report demonstrates the first reported successful treatment of a sinonasal hemangioma with intralesional bevacizumab. Intralesional bevacizumab confers an additional option for adjuvant treatment of sinonasal hemangiomas. Further evaluation of intralesional bevacizumab in the treatment of these tumors is warranted.

Sinonasal quality of life after endoscopic resection of malignant sinonasal and skull base tumors.

OBJECTIVES: Improvement in sinonasal quality of life (QoL) following sinus surgery has been well-documented across the literature. To our knowledge, only one series has evaluated long-term QoL in patients undergoing tumor resection, and that study demonstrated no improvement in rhinologic QoL following malignant tumor resection at 2-year follow-up. The objective of the present study was to evaluate QoL in the 2 years following endoscopic malignant tumor resection. METHODS: A prospective cohort study was performed, including patients with both malignant and benign sinonasal tumors in a tertiary academic medical center. Patients undergoing endoscopic tumor resection who had completed Sinonasal Outcome Test 22 (SNOT-22) questionnaires were included in the cohort. SNOT-22 questionnaires were administered preoperatively and over a 2-year follow-up period at clinic visits. Longitudinal linear mixed-effects regression was used to compare preoperative QoL to QoL over the 2 years following surgery. RESULTS: Among 145 patients included in this study, 64 had malignant tumors. There was a statistically significant improvement in SNOT-22 score from baseline to 2 years for patients with both malignant tumors (37.0, 95% confidence Interval [CI] 32.0-42.1 at baseline; 26.5 95% CI 20.8-32.2 at 2 years; P < 0.001) and benign tumors (26.5, 95% CI 21.4-30.4 at baseline; 12.9 95% CI 7.6-18.2 at 2 years; P < 0.001). CONCLUSION: In contrast to previously reported series, in this cohort endoscopic resection of sinonasal tumors appears to be followed by an improvement in QoL, which is sustained over a 2-year period. LEVEL OF EVIDENCE: 2b. Laryngoscope, 128:789-793, 2018.

A Case Report and Systematic Review of Eosinophilic Angiocentric Fibrosis of the Paranasal Sinuses.

OBJECTIVE: There is a paucity of literature discussing prognostic factors or comparing outcomes in eosinophilic angiocentric fibrosis (EAF). This review aims to analyze tumor and patient characteristics as possible prognostic markers and compare surgical approaches. METHODS: Systematic literature review and case report analyzing available cases of EAF located within the paranasal sinuses. RESULTS: The literature search yielded 39 articles meeting criteria for a total of 59 cases (including 1 from our institution). Median patient age was 46 years. The most common presenting symptoms were nasal obstruction (69%, n = 41) and change in external nasal appearance (39%, n = 32). The majority of cases (85%) were treated with surgical resection alone or in combination with medication. Of surgical patients, 62% underwent a complete resection with a recurrence rate of 20%. Median follow-up duration was 2 years. Endoscopic approach showed a significant positive correlation with complete resection ( P = .045). Patient sex ( P = .6), tumor location (range, P = .32-.98), lateral rhinotomy ( P = .26), septoplasty ( P = .84), and external rhinoplasty ( P = .28) were not significantly correlated with total resection. Insufficient sample size precluded calculation of predictors of recurrence following surgery. CONCLUSION: This review suggests that an endoscopic approach to EAF tumor is a viable option, frequently yielding complete resection.

Patient characteristics influencing the variability of distributed parameter-based models in DCE-CT kinetic analysis.

Kinetic parameter variability may be sensitive to kinetic model choice, kinetic model implementation or patient-specific effects. The purpose of this study was to assess their impact on the variability of dynamic contrast-enhanced computed tomography (DCE-CT) kinetic parameters. A total of 11 canine patients with sinonasal tumours received high signal-to-noise ratio, test-double retest DCE-CT scans. The variability for three distributed parameter (DP)-based models was assessed by analysis of variance. Mixed-effects modelling evaluated patient-specific effects. Inter-model variability (CVinter ) was comparable to or lower than intra-model variability (CVintra ) for blood flow (CVinter :[4-28%], CVintra :[28-31%]), fractional vascular volume (CVinter :[3-17%], CVintra :[16-19%]) and permeability-surface area product (CVinter :[5-12%], CVintra :[14-15%]). The kinetic models were significantly (P<0.05) impacted by patient characteristics for patient size, area underneath the curve of the artery and of the tumour. In conclusion, DP-based models demonstrated good agreement with similar differences between models and scans. However, high variability in the kinetic parameters and their sensitivity to patient size may limit certain quantitative applications.

[The analysis of 42 observations of paranasal sinus osteoma]. / Analiz 42 nablyudenii osteomy okolonosovykh pazukh.

The data on the incidence, localization, and histological structure of paranasal sinus osteoma (PSO) are reported along with the authors' experience in the diagnostics and surgical treatment of 42 patients presenting with this condition. The classification of the remote tumours based on their histopathological characteristics is considered. It is shown that 34 (80.9%) patients have osteomas of the compact type, 4 (9.5%) present with the tumours of the spongy type, and another 4 (9.5%) with the mixed type osteomas. It is maintained that the application of boron should be considered to facilitate the surgical removal of paranasal sinus osteomas.

Atypical presentations of sinonasal inverted papilloma: Surgical management and influence on the recurrence rate.

BACKGROUND: Sinonasal inverted papilloma (SNIP), classically, is a unilateral benign tumor of the nasal lateral wall. Numerous variations have been observed, depending on location, bilateral presentation, or association with nasal polyposis. OBJECTIVE: The aim of this work was to describe atypical presentations of SNIPs with their management specificities and to assess their influence on the recurrence rate in a large case series. METHODS: A retrospective single center study of 110 patients treated for SNIP. Atypical inverted papillomas were identified according to the following criteria: (1) unusual location (frontal, posterior, anterior), (2) bilateral involvement, and (3) association with nasal polyposis. Surgical management was detailed, and the influence of each atypical group on recurrence was assessed by using Kaplan-Meier survival curves and the log-rank test. RESULTS: Distribution of atypical presentations was as follows: frontal sinus localization (10.9%); posterior localization, including sphenoid sinus (9%); nasal anterior localization (3.6%); bilateral involvement (3.6%); and nasal polyposis association (10%). The surgical approach was endoscopic (74.5%), external (5.5%), or combined endoscopic and external (20%). Except for nasal anterior localization, all the groups were associated with a higher recurrence rate, without reaching statistical significance. CONCLUSION: Recurrence rates for these atypical presentations arise from their specific surgical challenges. The choice of the surgical technique is guided by tumor location and extension, and by the surgeon's experience; the main objective is a complete resection. The endoscopic endonasal approach is the most frequent procedure.

Role of Wnt signaling pathway in progression of sinonasal inverted papilloma to squamous cell carcinoma.

BACKGROUND: Sinonasal inverted papilloma (IP) is one of the most common benign tumors of the sinonasal area and malignant transformation has frequently been reported. However, the exact mechanism of the transition from benign lesion to malignancy is not known. The Wnt signaling pathway involves a network of multiple signaling glycoproteins that are known to play an important role in embryogenesis and carcinogenesis. OBJECTIVE: The purpose of this study was to evaluate the role of the Wnt pathway and signaling proteins in malignant transformation of IP to dysplasia and squamous cell carcinoma. METHODS: Expression of the Wnt signaling pathway proteins, including Wnt-1, beta-catenin, cyclin D1, and Dishevelled-1 (Dvl-1), were detected by immunohistochemistry by using 3-mm tissue core microarrays that consisted of 115 cores of IP tissue. Each of the IP cores was graded as I (prominent squamous metaplasia), II (inverted pattern), III (dysplasia), or IV (squamous cell carcinoma). The expression pattern of each protein and the correlation between the expression of each target protein and IP grade were evaluated. RESULTS: Membranous staining of beta-catenin showed a significant positive correlation with IP grade (ρ = 0.247, p < 0.001), as did staining of cyclin D1 (ρ = 0.365, p < 0.001), which showed a nuclear pattern and staining of Dvl-1 (ρ = 0.380, p < 0.001), which showed a membranous, cytoplasmic, and nuclear pattern. For Dvl-1, a nuclear expression pattern was more frequently observed in grade III and IV IP (p = 0.036). In the case of Wnt-1, cytoplasmic expression was observed; however, it did not show a significant correlation with IP grade (ρ = 0.141, p = 0.130). CONCLUSIONS: Wnt signaling proteins, including beta-catenin, cyclin D1, and Dvl-1, may play crucial roles in the malignant transformation of IP.

Endoscopic Resection of Sinonasal Tract Schwannoma: Presentation, Treatment, and Outcome in 10 Cases.

BACKGROUND: Sinonasal schwannoma (SNS) is a rare sinonasal tract tumor whose presentation is similar to that of other benign nasal tumors. Very few case series appear in the literature. This study aims to describe the presentation, treatment, and outcome of 10 SNS cases from 3 referral centers. METHODS: All SNS cases were analyzed retrospectively. Patient demographics, tumor data, surgical procedures, and outcome were recorded. RESULTS: Five males and 5 females (mean age, 49.1 ± 21.4 years) were included in the study. The tumor was on the left side in 9 patients and on the right side in 1. It originated in the middle turbinate in 4 cases, the ethmoid and sphenoid sinuses in 2 cases, and the nasal septum and infratemporal fossa in 1 case each. Tumor resection was done endoscopically in all cases, and there was no need for an external procedure, and 5 patients did not require a sinusotomy. All 10 patients remained disease-free after a mean follow-up of 65.3 months (range, 2-120 months). CONCLUSION: Safe and effective endoscopic resection of SNS is feasible. If the tumor is contained within the nasal cavity, simple tumor excision without sinusotomy can be enough to resect the tumor. In more extensive disease, a wider endoscopic approach may be needed, but an external approach was not necessary in this series.

[Neoplastic tumors in patients with unilateral inflammations qualified for endoscopic surgery]. / Guzy nowotworowe u chorych z jednostronnymi zmianami zapalnymi zakwalifikowanymi do operacji endoskopowych.

INTRODUCTION: The aim of this work was to analyze the occurrence and type of neoplasm in unilateral pathological lesions within the nose and paranasal sinuses in patients who underwent endoscopic surgery, in own material. MATERIAL AND METHODS: Between 2006 and 2012 2295 patients, including 1006 women aged 15-84 and 1289 men aged 17-87, were operated on due to diseases of paranasal sinuses in the Department of Otolaryngology and Laryngological Oncology. The patients underwent surgery after a medical interview, physical examination, 3D CT of paranasal sinuses and laboratory tests. The removed lesions were histopathologically investigated. The studied group included the patients with only unilateral lesions and histopathologically recognized neoplastic lesion. RESULTS: In the studied material, neoplastic lesions occurred in 9.8% cases, out of which 5.4% were inverted papillomas, 3.1% osteomas, and 1.5% malignant tumors. However, in the patients who were operated due to hypertrophic changes within paranasal sinuses, neoplasm was found in 1.3% cases, while inverted papilloma in 0.7%, osteoma in 0.4% and malignant tumors in 0.1%, which altogether amounts to 8 times higher occurrence of neoplastic lesions in unilateral changes. RESULTS: Unilateral changes within paranasal sinuses require highly inquisitive pre-operative diagnostics, peri-operative analysis and histopathological evaluation.

Late recurrence or secondary location? Towards a better understanding of the physiopathology of inverted papilloma.

INTRODUCTION: Inverted papilloma (IP) is the most frequent benign tumor of the nasal cavities. Recurrence is found in 12 to 14% of cases, mainly at the primary site, although also exceptionally in remote locations. The present paper discusses the physiopathogenesis of IP on the basis of a report of late second occurrence of IP at a remote location and a review of the literature. CASE REPORT: A man, who had undergone surgery in 1997 for ethmoid IP at the age of 56, presented 11 years later with nasal cavity IP at a second (frontal) location, discovered serendipitously during systematic follow-up and managed surgically without complication. DISCUSSION AND CONCLUSION: The physiopathology of nasal cavity IP remains unexplained. No reliable histologic or biological markers predict risk of recurrence or of malignant transformation. The sole treatment is total surgical resection. The risk of local recurrence, often due to incomplete resection, is well known, but that of a secondary location is less so, and regular very long-term follow-up is justified.

Imaging of the sinuses for functional sinus surgery using navigational guidance.

Advances in the understanding of mucociliary activity and pathophysiology of the nasal cavity and paranasal sinuses have revolutionized the surgical management of chronic and recurrent sinusitis. The development of functional endoscopic sinus surgery (FESS) can also be seen as the pioneer task in endoscopic surgery. Today, in more than 50% of all sinus surgeries FESS is being used. Inflammatory causes are the most common indication for surgery, which can be evaluated best preoperatively using (multi-detector) computed tomography. Even today there are further relevant developments of FESS that result in alleviation of the surgeon due to different degrees of automation. Using a preoperatively acquired navigational dataset helps choosing the optimal surgical strategy. Intraoperatively the navigational dataset is at present mainly used for localization of surgical tools. These newer operation methods demand for high-quality radiological imaging and evaluation, consisting of delineation of the local (inflammatory) process, detection of disease related complications and description of anatomical variations that may be important to the surgeon. Since patients with chronic recurrent sinusitis are relatively young on average and recurrent disease or further CT examinations are not uncommon, there should always be a focus on reduction of radiation exposure as much as possible. Technical advances like cone-beam tomography seem promising to further improve dose reduction as well as spatial resolution.

Wnt-pathway activation in intestinal-type sinonasal adenocarcinoma.

BACKGROUND: Intestinal-type sinonasal adenocarcinoma (ITAC) is an epithelial cancer of the sinonasal sinuses that shows histological similarity to colorectal cancer (CRC) and share chronic inflammation as a possible etiological factor. The Wnt-pathway is one of the most important tumourigenic pathways in CRC. The aim of this study was to investigate if the Wnt-pathway is activated in ITAC. METHODOLOGY: Protein expression profiles of E-cadherin, ß-catenin, c-myc and cyclin D1 were analysed by immunohistochemistry in 83 samples of ITAC, organized into tissue microarray blocks. RESULTS: Nuclear ß-catenin expression was observed in 31% of the cases and was twice as frequent in papillary/colonic ITAC compared to solid/mucinous subtypes. Loss of membranous ß-catenin staining occurred in 24% and loss of membranous E-cadherin in 6% of the cases and this was more prominent in mucinous types. Strong c-myc and cyclin D1 expression was observed in 30% and 4% of the cases, respectively. Nuclear ß-catenin expression was significantly related to poor clinical outcome, independent from established factors as tumour stage and histological type. CONCLUSION: The presence of nuclear ß-catenin in 31% of patients with ITACs indicated that in a subset of patients, the Wnt-pathway is active and conveys a worse prognosis.

Characterization of initial clinical symptoms and risk factors for sinonasal adenocarcinomas: results of a case-control study.

PURPOSE: Sinonasal adenocarcinoma is a rare cancer, frequently associated with occupational exposure to inhalable wood dust. Among the EU member States, Germany was reported to have the highest number of exposed workers. Location and long latency make early diagnosis difficult. This case-control study was aimed at assessing potential risk factors and at characterizing initial clinical symptoms, both serving as matrix to identify persons at increased risk and to improve management of this cancer. METHODS: Of 58 patients, 31 diagnosed with sinonasal adenocarcinoma (cases) between 1973 and 2007 were identified and underwent standardized interview on clinical data. A total of 85 patients diagnosed over the same period with carcinoma of the oral cavity served as controls. RESULTS: The ethmoid was confirmed as the predominant site of adenocarcinoma associated with wood dust exposure, whereas the nasal cavity was most commonly affected in patients denying any exposure to wood dust. Cases were significantly engaged in mainly woodworking occupations compared to controls. The main initial clinical symptoms were nasal obstruction 6 months (71%) and epistaxis 4 years (41.9%) prior to diagnosis. Hardwood dust from beech and oak proved to be the most common occupational exposure, with a mean exposure period of 32.3 years and a latency of 43.5 (34-58) years. CONCLUSIONS: Our investigation confirms the importance of regular screenings for persons exposed to wood dust even years after the end of occupational or private exposure. Banal clinical symptoms such as epistaxis and nasal obstruction might be predictive, requiring early and thorough investigation.

Juvenile nasopharyngeal angiofibroma recurrence associated with exogenous testosterone therapy.

BACKGROUND: Juvenile nasopharyngeal angiofibromas (JNAs) are rare benign lesions that express hormonal receptors. This report describes a recurrence of a JNA 20 years after excision associated with exogenous testosterone therapy. METHODS: A 36-year-old man developed a sphenoid mass 20 years following resection of a JNA, shortly after initiating exogenous testosterone therapy for symptomatic low endogenous testosterone. RESULTS: The mass was subsequently excised and was histologically consistent with a JNA. The patient resumed his testosterone therapy postoperatively. Repeated imaging has demonstrated no recurrence after 3 years. CONCLUSION: This unique case adds further evidence to the role of testosterone in the pathogenesis of JNAs. Exogenous testosterone can cause tumor regrowth at any time, even decades following treatment. The patients with a history of JNA, even those without recurrence for years, should weigh the risk of recurrence before the use of exogenous testosterone.