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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.

​: ​The study aims to discuss a case of an uncommonly sited rare tumour causing nasal obstruction and a literature review of angioleiomyomas in the nose. A 64-year-old woman presented with blockage of the right nostril, associated with a visible swelling on the undersurface of the alar cartilage, in the lateral wall of the nasal cavity. Anterior rhinoscopy highlighted a 1 cm cystic lesion, with areas of firmness, present at the mucocutaneous junction with a broad base. It was not attached to the underlying cartilage. There was no ulceration or bleeding on palpation. Nasal endoscopy did not show any extension further and the rest of the nasal cavity was clear. Endonasal resection of the tumour using a two-handed technique. This paper highlights not only the importance of considering angioleiomyomas within a differential diagnosis of nasal obstructions but also the uncommon site. ​., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

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.

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.

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., (© 2017 The American Laryngological, Rhinological and Otological Society, Inc.)

The paper describes a case of nasal glial heterotopia in a 10-month-old girl with a mixed (intranasal and subcutaneous) localization, which is accompanied by the divergence of the nasal bones. Histological examination supplemented by immunohistochemical reactions with antibodies to vimentin, S100 protein, neuron-specific enolase, as well as Ki-67 and smooth muscle actin confirmed the neural nature of the tumor. Fields of mature astrocytic glia including individual cells with neuronal differentiation were found among the fibrous and fibrovascular tissues. The paper provides a brief overview of the discussed pathology.

The objective of the present work was to present the results of the clinical analysis of the patient presenting with natural killer (NK)/T-cell lymphoma of the nasal type. We undertook the analysis of the medical documentation concerning the case of interest. It was shown that the development of progressive perforation of the nasal septum and the pronounced destructive changes in the intranasal and adjacent structures following the endonasal surgical interventions made necessary differential diagnostics between the condition under consideration and certain latent disorders (such as Wegener's granulomatosis, leprosy, syphilis, leishmaniasis, dirofilariasis tuberculosis, etc.). The study has demonstrated that the negative results of the analysis imply the necessity of special attention to the possibility of development of oncological diseases including hematological disorders (e.g. NK/T-cell lymphoma) and the repeat careful follow-up examination of the patients by the experienced experts.

Nasal lymphoma is the most common nasal tumor in cats and is generally a solitary and radiosensitive tumor. We retrospectively evaluated the response to radiation and survival time in relation to apoptosis and Ki-67 indices in feline nasal lymphomas treated with radiation therapy. The apoptotic and Ki-67 indices were evaluated with TUNEL and immunohistochemical staining in 30 biopsy tissues that were taken before any treatment. These two indices were compared, and differences between different treatment response groups were analyzed. The correlation between the median survival times (MST) and the indices was estimated using the Kaplan Meier method, and statistical differences between survival curves were analyzed using a log-rank method. With regard to apoptotic index, a statistical difference was observed between the samples taken from cats with complete response and stable disease (1.22% vs. 0.45%; P=0.045). The Ki-67 index in cats with both complete response and partial response was significantly higher than in cats with stable disease (44.4% and 39.6% vs. 16.3%; P<0.001 and P=0.008, respectively). The cats with a high level of apoptosis (>0.9%) nasal lymphoma were not significantly prolonged MSTs (P=0.202), however, high Ki-67-positive (>40%) cats experienced a statistically significant relationship with longer survival time (P=0.015). Our results indicate that spontaneous apoptotic and Ki-67 indices are strong predictors for response to radiation therapy in feline nasal lymphomas.

Introduction: Esthesioneuroblastoma is a rare tumour of the sino-nasal tract. One-third cases present with intracranial extension. However, treatment options are limited for such cases., Methodology: We herein report a case with large intracranial extension treated with Induction chemotherapy, de-bulking surgery, and image guided intensity modulated radiotherapy., Results: The patient was treated with IGIMRT technique to a dose of 64 Gy in 32 fractions. Cone bean CT verification was done twice a week to eliminate set up error. The patient achieved complete resolution of the disease and was disease free 6 months after completion of treatment., Conclusion: IGIMRT even after a de-bulking surgery may help to achieve long-term disease control for patients with large intracranial extension with minimal morbidity.

Objectives: The goal of this study was to review the main lesion types of the nasal skin and appropriate treatment strategies rather than to present a comprehensive list of all diseases that affect the skin that can involve the nose., Methods: We reviewed the main nasal skin lesion types and available treatment strategies. Nasal skin lesions were classified as benign, premalignant, or malignant., Results: Benign lesions of the nose include nonmalignant tumoral lesions (i.e., freckles, comedo, adenoma sebaceum [Pringle disease], hydrocystoma, fibrous papules, sebaceous hyperplasia, and rhinophyma), autoimmune and inflammatory conditions (i.e., pemphigus, sarcoidosis, systemic lupus erythematosus, facial eosinophilic granuloma, rosacea, herpes zoster infection, leishmaniasis, and leprosy), and vascular lesions (i.e., telangiectasis, hemangioma, and spider nevus). Premalignant lesions are actinic keratosis and keratoacanthoma; and malignant tumors are melanoma, basal cell carcinoma, and squamous cell carcinoma. Regardless of whether or not they are malignant, all facial lesions can yield significant cosmetic discomfort that should be evaluated carefully before commencing any curative or corrective intervention. In general, benign lesions are treated with dermabrasive modalities, such as trichloroacetic acid, phenol, salicylate, and laser ablation. Electrocautery, cryosurgery, and surgical excision are also used, although these methods may result in scar formation, which can sometimes be more problematic than the original lesion itself., Conclusion: Any disease that affects the skin, especially those diseases that are triggered by ultraviolet exposure, can involve the face and nose. Cosmetic defects due both to the lesion itself and the intervention must be discussed with the patient, preferably in the presence of a first-degree relative, before commencement of treatment. As a result of heterogeneity of skin lesions of the nose, appropriate education of general practitioners as well as otorhinolaryngologists is mandatory.

Olfactory neuroblastoma (ONB) is a rare cancer originating in the olfactory epithelium of the nasal vault. The recurrence rate of ONB is high, as the standard treatment of surgery followed by radiotherapy and/or chemotherapy is usually unsuccessful. The use of targeted therapy based on individual genomic variations after cancer relapse has not been reported. Here, we present the case of a 44-year-old man who was diagnosed with recurrent ONB and treated with a regimen developed using whole exome sequencing. Potential targets were first identified and then matched to appropriate drugs. Gene mutations in the genes encoding EGFR, FGFR2, KDR, and RET were discovered in the patient's tumor tissue by whole exome sequencing and the patient was treated with a combination of the targeted drugs cetuximab and sunitinib. Five days after treatment, enhancement magnetic resonance imaging showed a 65% reduction in tumor size, and the Visual analog scale headache scores went down to 2/10 from 10/10. Repeat imaging at 1 month showed a complete response.This study represents the first demonstration of an effective personalized treatment of ONB by targeted drugs, and sheds light on how precision medicine can be used to treat recurrent ONB that fails to respond to routine tumor resection, radiotherapy, and/or chemotherapy.

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.

The expression profiles of 14-3-3β and θ isoforms, known to exert both oncogenic and antiapoptotic effects, were assessed in different entities of nasal pathophysiology. Flow cytometry and immunohistochemistry were used on paraffin-embedded sections of 51 inverted papillomas (IP), 26 nasal polyps (NP), 9 polyps with IP (NPIP) and 10 specimens of normal epithelium (NE). 14-3-3β expression was significantly upregulated in IP as compared with both NP (p=0.015) and NE (p=0.002). 14-3-3β was also increased in NPIP as compared with NE (p=0.008). 14-3-3β cytoplasmic staining was more pronounced in basal cells of the respiratory epithelium although serous glands and the vascular system were often positive as well. High 14-3-3β immunopositivity in IP patients concurred with increased proliferative activity shown by PCNA immunostaining (p=0.04). Expression of 14-3-3θ was also found increased in IP and NPIP patients, compared to NP (p=0.005, p=0.002 respectively) and NE (p=0.004 and p=0.001 respectively). 14-3-3θ cytoplasmic immunopositivity was detected in columnar epithelium, particularly in basal and subluminal cells, whereas no immunoreactivity was observed in NP and NE. Our results demonstrate differential expression of 14-3-3β and θ isoforms in sinonasal pathophysiology, supporting their implication, respectively, in the proliferative and inflammatory process engaged in the formation of IP., (Copyright © 2013 Elsevier GmbH. All rights reserved.)

Nasopharyngeal carcinoma (NPC) is one of the most common cancers originating in the nasopharynx, and chemoresistance is an essential aspect of NPC chemotherapy failure. Sorcin has been implicated in multidrug resistance (MDR) of many types of human tumor. However, the effect and mechanism of Sorcin in MDR of human NPC is not fully clear. In this report, we silenced Sorcin in human NPC CNE2/DDP cells, and explored the role of Sorcin in MDR reversal. The results showed an increased cytotoxicity of cisplatin and intracellular accumulation of Rhodamine-123 and glutathione depletion in Sorcin silencing CNE2/DDP cells. We also found a decreased messenger RNA and protein expression of multidrug resistance gene (MDR1), multidrug resistance-associated protein (MRP1), excision repair cross-complementing gene 1 (ERCC1), glutathione S-transferase-π (GST-n), RhoE, Bcl-2, and Survivin in Sorcin silencing CNE2/DDP cells. The increased expression of PTEN and decreased expression of p-Akt and NF-κB suggested that the key cellular signaling pathways were triggered by Sorcin silencing. We concluded that Sorcin silencing would contribute to establish a potent target point for MDR reversal., (Copyright © 2013 Wiley Periodicals, Inc.)

Objective: To discuss the role of bilateral adrenalectomy in Cushing syndrome, as illustrated in a case of severe hypercortisolism that was unresponsive to combination agent medical therapy., Methods: We report the clinical, laboratory, imaging, and pathologic findings in a patient with ectopic Cushing syndrome attributable to an adrenocorticotropic hormone (ACTH)-secreting neuroblastoma. In addition, we provide a literature review regarding olfactory neuroblastoma and discuss current and emerging therapeutic options for Cushing syndrome., Results: A 59-year-old man presented with nasal congestion and neck swelling and was noted to have hypokalemia, hypertension, and hyperglycemia. A nasal biopsy demonstrated a poorly differentiated carcinoma with neuroendocrine features. He was subsequently diagnosed as having ACTH-dependent Cushing syndrome, but despite high-dose combination medical therapy, his condition rapidly deteriorated. Urgent bilateral adrenalectomy provided rapid control of the hypercortisolism, and the patient was later able to undergo an uncomplicated total macroscopic resection of his locally metastatic primary tumor., Conclusion: This report describes the challenges in the diagnosis and management of ACTH-dependent Cushing syndrome and highlights the important role that bilateral adrenalectomy can still have in patients with severe hypercortisolism causing life-threatening complications.

Background: Osteopontin (OPN) is involved in cell survival, immunity, and tumor progression. The overexpression of OPN has been proposed as a biomarker of progression and metastasis for several tumor types, but it is still unclear whether it is up-regulated in sinonasal inverted papilloma (SIP)., Methods: We enrolled 33 subjects with SIP and 15 normal controls to determine the importance of OPN and vascular endothelial growth factor (VEGF) in SIP. Using immunohistochemistry, quantitative polymerase chain reaction, and enzyme-linked immunosorbent assay, we examined the distribution, mRNA expression, and protein levels, respectively, of OPN and VEGF. We then correlated these values with clinical severity., Results: The immunostaining levels for OPN and VEGF were significantly increased in SIP tissues compared with control tissues (p < 0.05), as were their mRNA expression and protein levels (p < 0.05). The correlation between OPN and VEGF expression and the clinical stage of SIP was significant (p < 0.05)., Conclusion: Our findings indicate that OPN and VEGF were both overexpressed in the analyzed SIP tissues and were associated with clinical severity, suggesting that the OPN-VEGF axis might contribute to tumor progression by enhancing angiogenesis. Therefore, OPN may serve as a potential therapeutic target for preventing SIP progression and recurrence.

Hemangiopericytoma is an unusual tumor that arises from vascular smooth muscle cells. It is mainly experienced in 4th to 5th decades of life and soft-tissue masses are frequently located in lower extremity, pelvis, and retroperitoneum. A 29-year-old man presented with a mass lesion in the right nasal cavity associated with shortness of breath as a result of nasal obstruction. An incisional biopsy was reported as sinonasal type hemangiopericytoma. The initial staging F-18 flourodeoxyglucose PET/CT successfully demonstrated severe hypermetabolism in the primary tumor location and mild-to-moderate hypermetabolism in the right upper jugular lymph node.

Malignant tumors of the paranasal fossae and sinuses represent 0.2% to 0.8% of all malignant tumors in the body and 3% to 6% of other head and neck tumors. It is possible to distinguish 4 different histologic types of sinonasal malignant tumors: squamous carcinoma, adenocarcinoma, adenoid cystic carcinoma, and sinonasal undifferentiated carcinoma. According to the WHO classification, there are 2 main types of adenocarcinomas: intestinal-type (ITAC) and nonintestinal type. ITACs are generally connected with professional exposure to wood and leather dust. The metalloproteinase 9 (ADAM-9) is a type 1 transmembrane protein that has been associated with cancer development and metastases. Our case of poorly differentiated sinonasal ITAC showed moderate to strong cytoplasmic positivity for ADAM-9 in association with moderate membrane staining for c-erbB-2 oncoprotein. In our opinion, the expression of these proteins could explain the tumoral growth and together they could represent the most interesting targets when designing any tumor treatment scheme.

Background: It has been suggested that sinonasal inverted papilloma (IP) can progress to squamous cell carcinoma (SCC); however, mechanisms of malignant transformation are not fully understood. This study investigated alterations in the expression of cell cycle-related proteins in a multistep process of malignant transformation of IPs., Methods: The expression of cell cycle-related proteins, including p53, p21, p16, and p63, was evaluated by immunohistochemistry in 21, 56, 7, and 18 cases of nasal polyps, IPs, IPs with dysplasia, and IPs with SCC, respectively. Furthermore, we determined the possible correlation between the expression of these proteins and clinicopathological variables in patients with IPs with SCC., Results: Expression of p53 was found only in 8 of 18 IPs with SCC (44.4%). The frequency of p21 positivity was significantly higher in IPs with dysplasia (71.4%) and IPs with SCC (77.8%) compared with nasal polyps (0%) and IPs (12.5%). A p16 positivity was observed in 82.1% of IPs and 57.1% of IPs with dysplasia, whereas 83.3% of IPs with SCC showed an apparent loss of p16 protein expression. A p63 positivity was found in all specimens, but the percentage of positive cells was significantly increased in IPs with dysplasia and IPs with SCC compared with nasal polyps and IPs. There was no significant correlation between the expression of these proteins and clinicopathological variables, such as tumor stage, histological differentiation, and the proportion of malignant areas in patients with IPs with SCC., Conclusion: Alteration of cell cycle-related proteins may contribute importantly to the malignant transformation from IP to SCC.

Context: Primary cutaneous lymphoma is an uncommon, extranodal lymphoma, and it is usually more indolent with a better prognosis than its histologically similar systemic counterpart is., Objectives: To illustrate the clinicopathologic features of cutaneous lymphomas in Taiwan and to compare the relative frequencies of subtypes of cutaneous lymphoma among different geographic areas., Design: A total of 56 patients with cutaneous lymphomas were retrospectively collected and were reclassified according to the 2005 World Health Organization and the European Organization for Research and Treatment of Cancer and the 2008 World Health Organization classifications. The data were compared with those from other studies for different geographic areas., Results: Thirty-one (55%) tumors were primary cutaneous lymphomas, and twenty-five (45%) tumors were secondary or concurrent cutaneous lymphomas. Among primary cutaneous lymphomas, 23 cases (74%) were T-cell or natural killer-cell lymphomas, and 8 cases (26%) were B-cell lymphomas. The most common types were extranodal natural killer/T-cell lymphoma, nasal type, and primary cutaneous peripheral T-cell lymphoma, unspecified (5 cases each; 16%). In contrast with other primary cutaneous B-cell and T-cell lymphomas, either primary or secondary extranodal cutaneous natural killer/T-cell lymphomas, nasal type, had extremely poor prognoses (1-year overall survival, 0%)., Conclusions: This study showed that the frequency of subtypes of primary cutaneous lymphoma varied in different geographic areas. Compared with the Western countries, there was a higher frequency of extranodal natural killer/T-cell lymphoma, nasal type, and a lower frequency of mycosis fungoides in Taiwan. Extranodal natural killer/T-cell lymphoma, nasal type, also had an extremely poor prognosis compared with other lymphomas.

Background: Contact endoscopy (CE) was initially described as a method used in the analysis of uterine and vocal folds histology. The first nasal cavity CE studies achieved promising results regarding its use for the differentiation between benign and malignant lesions, considering that biopsy might cause some complications, especially bleeding. This study described and compared the findings of CE on inverted papilloma and nasosinusal squamous cell carcinoma (SCC) and tested the effectiveness of this exam as a noninvasive method for in vivo differentiation between these tumors., Methods: The patients included in this study were divided into group A, patients diagnosed with inverted papilloma, and group B, patients diagnosed with SCC. CE results were compared among themselves. CE images were presented to examiners not experienced with the method., Results: Twenty-two patients were examined, 13 in group A and 9 in group B. The main relevant differences in CE findings between those two groups were corkscrew vessels, presence of mitoses, keratinization and nuclear pleomorphism in carcinoma, and vacuolated cells in papilloma. The examiners were capable of defining the diagnosis of these nasal tumors only based on CE images., Conclusion: CE may be a useful noninvasive exam to be used in the in vivo diagnosis of inverted papilloma and nasosinusal SCC, which may enable better preoperative planning.

Extracranial meningiomas are rare tumors that usually represent extensions from intracranial lesions. Here, we report a case of primary meningioma of the nasal septum. A 60-year-old man presented with nasal obstruction and postnasal drip. On examination he had a mass in the nasal cavity. Computed tomography studies revealed absence of intracranial extensions. The mass was successfully excised and the histologic diagnosis of meningioma was established. This report documents what we believe to be the first case (in English medical literature) of an extracranial meningioma, involving the nasal septum. The clinicopathologic features and pathogenesis of the primary nasal meningioma is briefly discussed.

Background: Epidemiological and clinical data implicate that in patients with cancer, continued smoking causes progression of cancer growth and resistance to therapy. The carcinogens possess the ability to block apoptosis, an important mechanism in the development of tumors and resistance to chemotherapy. We previously showed that nicotine enhances growth and proliferation in lung cancer. However, the effects of nicotine, a tobacco carcinogen that inhibits apoptosis, have not been studied before in nasal epithelial carcinoma (NC). In this study, we sought to determine the effects of nicotine on chemotherapy-induced apoptosis in human NC., Methods: Primary human NC cells were grown per protocol, treated with combination chemotherapy, and the apoptosis was assessed by TUNEL (terminal deoxynucleotidyl transferase biotin-dUTP nick end labeling) and DNA fragmentation assays. The regulation of mitogen-activated protein kinase (MAPK) and protein kinase B (AKT) signal transduction pathway was examined by real time quantitative polymerized chain reaction, and immunofluorescent staining assays., Results: Combination chemotherapy with cisplatin (35 microM) plus etoposide (20 microM) caused a significant increase in NC apoptosis compared with single agent alone, and nicotine, in part, inhibited chemotherapy-induced apoptosis in NC. Furthermore, nicotine induced activation of AKT and MAPK pathways, while inhibition of MAPK using U0126 and AKT by phosphatidylinositol 3-kinase inhibitor, LY294002, in part, blocked the antiapoptotic effects of nicotine against cisplatin and etoposide-induced apoptosis in NC., Conclusion: Nicotine inhibits chemotherapy-induced apoptosis in NC via the AKT and MAPK-mediated signaling pathways. We speculate that nicotine may play a role in oncogenesis and resistance to cancer therapy in NC.

Background: Preliminary reports support the role of endoscopic techniques in the management of selected sinonasal malignancies. The objectives of this review are (1) to assess outcomes for patients undergoing definitive endoscopic tumor extirpation and (2) to elucidate the role of endoscopy in the management of sinonasal malignancies., Methods: Retrospective review of patients with sinonasal malignancy managed via endoscopic techniques from September 1998 to December 2007 was conducted., Results: Sixty-one patients were identified (38 men and 23 women) with a mean age of 59 years. The five most common pathologies were squamous cell carcinoma (21), melanoma (10), esthesioneuroblastoma (8), adenocarcinoma (7), and sinonasal undifferentiated carcinoma (3). Sixty-nine endoscopic procedures were performed and categorized according to surgical intent: 57 curative resections (82.6%), 8 palliative resections (11.6%), and 4 tumor mappings (5.8%). Adjuvant chemotherapy and/or radiation therapy was used in 29 of the 50 patients (58%). For those patients undergoing definitive treatment, the disease-free and overall survival rates were 69.2 and 84.6% at mean of 34.5 and 42.7 months, respectively. The local and regional recurrence rates were 17 and 10%, respectively., Conclusion: The endoscope is a versatile tool for management of sinonasal malignant neoplasms. Although the role was once just limited to biopsy, endoscopic approaches allow for definitive resection for curative intent and palliative resection for patient comfort with acceptable survival. Moreover, endoscopy facilitates three-dimensional tumor mapping and posttreatment surveillance in patients undergoing definitive chemoradiation.

Background: At present, transnasal endoscopic surgery is considered a viable option in the management of small-intermediate size juvenile angiofibromas (JAs). The authors critically review their 14-year experience in the management of this lesion to refine selection criteria for an endoscopic approach., Methods: From January 1994 to May 2008, 46 patients were treated by a pure endoscopic resection after vascular embolization (87%). The lesions were classified according to Andrews (Andrews JC, et al., The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach, Laryngoscope 99:429-437, 1989) and Onerci (Onerci M, et al. Juvenile nasopharyngeal angiofibroma: A revised staging system, Rhinology 44:39-45, 2006) staging systems. All patients were followed by regular endoscopic and magnetic resonance imaging (MRI) examinations., Results: Lesions were classified as follows: stage I, n = 5; stage II, n = 24; stage IIIa, n = 14; stage IIIb, n = 3 according to Andrews classification system; stage 1, n = 9; stage II, n = 12; stage III, n = 26 according to Onerci's system. Unilateral blood supply was detected in 39 (85%) cases. Feeding vessels from the internal carotid artery (ICA) were also reported in 14 (30%) patients. Intraoperative blood loss ranged from 250 to 1300 mL (mean, 580 mL). In four (8.7%) cases, suspicious residual disease was detected by MRI. In one patient, a 1-cm persistent lesion was endoscopically removed because septoplasty was required and a slight increase in size was noticed. The other three lesions, all located in the root of the pterygoid plate, are nearly stable in size and are currently under MRI follow-up., Conclusion: The improvement of surgical instrumentation and the experience acquired during a 14-year period have contributed to expanding the indications for endoscopic surgery in the management of JAs. Even stage III lesions may be successfully managed, unless the ICA is encased or if it provides an extensive blood supply. An external approach may be required when critical structures such as the ICA, cavernous sinus, or optic nerve are involved by lesions that are persistent after previous treatment; such a situation may prevent safe and radical dissection with a pure endoscopic approach. Better understanding of the factors influencing the growth of residual lesions is needed to differentiate those requiring re-treatment from those which can be simply observed.

Background: This study reviewed the published outcomes literature on endoscopic or endoscopic-assisted treatment of sinonasal and skull base adenocarcinoma (SSBA). A systematic literature review was performed., Methods: A PubMed search of English language articles on endoscopic or endoscopic-assisted SSBA was performed. Each article was examined for patient data and outcomes for analysis., Results: Sixteen articles (150 retrospective cases) using endoscopic or endoscopic-assisted SSBA were identified. Three (19%) reported at least 15 cases of adenocarcinoma with 5-year survival ranging from 53 to 83%. In all identified studies, five (31%) reported adenocarcinoma-specific survival statistics and eight (50%) provided complete patient information. Adjuvant therapy was used in 12 studies (75%), with details outlined in 6 (38%) articles, accounting for 52 of 74 cases (70% of those patients and 35% overall). Adenocarcinoma-specific follow-up was reported in only nine studies (56%); four of these nine articles had a median/mean follow-up time of <3 years. Six articles (38%) used American Joint Committee on Cancer guidelines and four articles (25%) used International Union Against Cancer guidelines. The remaining six articles (38%) did not report on the stage of treated tumors. Four articles (25%) reported length of hospital stay and eight (50%) reported complication data. Three studies comprising 61 cases (41% of overall patients) recorded disease status, tumor stage, site involvement, and complication rates for each patient., Conclusion: From the existing body of data, one can not conclude that endoscopic methods are superior or inferior to open methods, but there is growing evidence that this is an efficacious technique. Future studies should have emphasis on detailed, prospective reporting.

Background: The aim of this study was to describe treatment results in patients with sinonasal inverted papilloma and to compare recent classification staging systems., Methods: We performed a retrospective study of 79 patients with primary paranasal sinus inverted papilloma from 1978 to 2008., Results: The median age at diagnosis was 59 years. There were 68 men (86%) and 11 women (14%). Sixty inverted papilloma (76%) were located in the ethmoidal sinus complex, 16 (20%) were in the maxillary sinus, 2 were in the sphenoid sinus (3%), and 1 was in the septum (1%). Malignancy was simultaneously associated in four cases (5%). Intracranial extension was found in five patients (6%) and we did not find orbital content involvement. The external approach was used in 22 cases (28%) and 57 patients were treated with endoscopic nasal resection (72%). The overall recurrence rate in the total population was 21%. Recurrences were observed in 7 cases (32%) in the group of patients who received external approach and in 10 (17%) patients in the endoscopic group. Recurrences according to the Krouse system were 0% in T1 stage, 16% in T2 stage, 25% in T3 stage, and 60% in T4 stage (p = 0.05). Recurrences for groups A, B, and C stages using the Cannady staging system were 12, 26, and 60%, respectively (p = 0.039). Recurrences according to Han's system were 19% in both T1 and T2, 20% in T3, and 60% in T4 (p > 0.05)., Conclusion: Our results support the endoscopic approach as the treatment of choice when feasible in patients with inverted papilloma, combined in selected cases with external approaches. The Krouse and Cannady systems provided a good distribution of patients according to local control.

Background: The etiology of sinonasal inverted papilloma (IP) is unknown. This study was designed to evaluate the possible risk factors associated with IP Methods: This is a case-control epidemiology study in a tertiary referral center. Fifty patients with IP and 150 matched controls were interviewed using a questionnaire on suspected risk factors. Univariate analysis of the risk factors and calculation of the matched odds ratios, the corresponding 95% CIs, and p values was performed. Significant risk factors were further studied using conditional logistic regression analysis., Results: Outdoor and industrial occupations were associated with IP. Tobacco smoking, drinking alcohol, history of allergic rhinitis, sinusitis, nasal polyp, non-sinonasal papilloma and non-sinonasal malignancy were not significant factors., Conclusion: Outdoor and industrial occupations were associated with IP and may be potential risk factors. Future studies are warranted to further evaluate the individual type of occupation and chemical involved.

Nasal obstruction is one of the most common complaints evaluated by the otolaryngologist. The differential diagnosis is broad, ranging from benign reversible causes to life-threatening diseases. In addition, patients may suffer from a combination of etiologies, further confusing the diagnosis. Surgical management is directed at the underlying origin of obstruction. The clinician therefore must use a careful history and physical examination as well as appropriate sinonasal imaging in accurately identifying the causes of nasal obstruction. To further illustrate these principles, we present two interesting cases of patients presenting with nasal obstruction.

Background: Birhinal testing of odor identification will not allow the detection of unilateral olfactory loss. The aim of the presented study was to evaluate side differences of odor identification in large groups of healthy subjects and in patients with nasal symptoms., Participants and Method: Self-assessment of olfactory function and evaluation of olfactory function by means of a validated test were performed in 479 healthy subjects, in 765 patients with chronic rhinosinusitis (CRS), and in 53 patients with a tumor. A 12-item odor identification test ("Sniffin' Sticks") was used to evaluate olfactory function separately for each nostril., Results: Fifteen percent of the healthy subjects demonstrated side differences in the identification of at least 3 out of 12 odors. Healthy elderly subjects showed larger side differences in identification of odor than younger ones; a general difference between odor identification with the right or left nostril was not found. Both CRS patients and patients with a tumor had larger side differences than healthy subjects. Only 20% of the patients with a tumor complained about impaired olfactory sensitivity, but more than 75% of them showed deficits in olfactory tests., Conclusion: Side differences of odor identification of 25% or greater should give reason for further investigation. Future studies are needed to investigate whether side differences in healthy subjects are a predicator of a higher risk for general olfactory loss.

Various growth factors are expressed in nasal polyps, and some of these have been suggested to play a role in polyp formation. A potential relation between growth factor expression and polyp recurrence, however, is undetermined. Basic fibroblast growth factor (bFGF) is expressed in mononuclear cells, as well as in endothelial and epithelial surface and gland cells of nasal polyps. To determine whether bFGF may play a role in the recurrence of nasal polyps, the present study aimed at a comparison of bFGF expression in recurrent versus non-recurrent polyps. Further, the expression in polyps from asthmatic patients was compared with that from non-asthmatics. Thirty patients with newly diagnosed nasal polyposis were included. Polypectomy was performed at entry to the long-term follow-up study. Fifteen patients only had one polypectomy (no recurrence group, with a median observation time of 81 months). Fifteen patients had a median of 6.4 polypectomies (multiple recurrence group, with a median observation time of 108 months). Five of nine patients with asthma belonged to the non-recurrence group and four to the recurrence group. The polyp from the entrance polypectomy was examined for expression of bFGF by immunohistochemistry, using a polyclonal antibody. A masked semi-quantification of staining intensity was performed in recurrent versus non-recurrent polyps, as well as in asthmatics versus non-asthmatics. bFGF expression was seen as varying staining of the polyp surface and gland epithelium, as well as of some mononuclear cells and some fibroblast-like cell profiles in the polyp stroma. Vascular endothelium was labeled occasionally. Semi-quantification of the staining intensity showed no significant differences between recurrent and non-recurrent polyps, or between asthmatics and non-asthmatics. We conclude that the level of immunohistochemical expression of bFGF in recurrent and non-recurrent nasal polyposis is equivalent. Thus, the level of bFGF expression in the primary polyp can not predict a subsequent recurrence. The expression of bFGF is not up-regulated in patients with asthma. Further studies are needed to determine a potential role of bFGF in nasal polyposis, with special reference to different stages of polyp formation and growth.

Background: Despite existing clinical and histopathological evidence, the role of inflammation in the pathogenesis of inverted papilloma (IP) is not well understood The goal of this study was to describe the inflammatory cell component present in sinonasal papilloma (SP), with the intention of further defining the existence of inflammation in IP and perhaps gaining insight into IP pathophysiology., Methods: Computerized database analysis was performed to identify all patients with SP who underwent surgery at the Cleveland Clinic Foundation between 1995 and 2001. Histopathological features of all SP were reviewed and semiquantitative analysis of the inflammatory cells present was performed. IP was histopathologically graded into four categories by the extent of inflammatory infiltrate and cellular atypia. Statistical analysis of the inflammatory cell component present in the epithelial layer of exophytic papilloma and IP was performed., Results: SP was classified into three types: cylindrical papilloma (5% [2/41]), exophytic squamous papilloma (34% [14/ 41]), and IP (61% [25/41]). Twenty-eight instances of IP in 25 patients were identified. Altogether, 11% were grade I (3/28), 54% were grade II (15/28), 25% were grade III (7/28), and 11% were grade IV (3/28). The inflammatory cell population was significantly greater in IP compared with other SPs and greater in grades I and II IP when compared with grade III and IV IP., Conclusion: Inflammatory cells were identified as a significant cell population in IP, whereas it was less commonly encountered in other forms of SP. The proposed IP staging system may serve as the foundation for improved understanding of IP and, ultimately, may help to predict recurrence or apparent malignant transformation.

This report details clinical, histopathological and immunohistochemical findings in 18 cats with chronic nasal disease diagnosed as nasal lymphoma. Eight of the cats were female and 10 were male, with a median age of 10.5 years (range 7-14 years). Three of the cats were Siamese, one was Burmese, and the rest were non-pedigree. The duration of clinical signs before referral ranged from 30 to 540 days (median 88.5 days). The most common clinical signs were nasal discharge, stertor and sneezing. Nasal radiographs were abnormal in 14/16 cases examined. Abnormal masses were detected endoscopically in 13/18 cases. Nine cats received multi-agent chemotherapy or radiation therapy, or both, with survival times ranging from 14 to >541 days. Biopsy material from these 18 cats was examined by light microscopy, and serial sections were subjected to immunohistochemical labelling for the T lymphocyte marker CD3 and the B lymphocyte marker CD79a. In 13 tissues, expression of class II molecules of the major histocompatibility complex and the myelomonocytic antigen MAC387 was also determined. Twelve of the tumours were classified as diffuse large B-cell lymphomas, four as lymphoblastic B-cell lymphomas, and one as a follicular B-cell lymphoma. The tumour cells within these lesions all expressed CD79a, and (where tested) most also expressed MHC class II. One tumour was an anaplastic large cell neoplasm, in which the neoplastic cells expressed MHC class II alone in the absence of either lymphoid marker. There was a variable infiltration of reactive small T lymphocytes into these tumours, and zones of necrosis within the tumour tissue were sometimes heavily infiltrated by MAC387+ phagocytic cells.

Transcription of natural killer (NK) cell antigen receptors (NKRs), such as CD94, NKG2, and killer immunoglobulin-like receptors (KIRs), is developmentally regulated and clonally distributed. We have shown a restricted KIR repertoire (rKIR-R) without monoclonal T-cell receptor rearrangement (mTCR-R) supports a NK lineage in nasal-type extranodal NK/T-cell lymphoma (NTENL) but does not correlate with clinical outcomes. Developing NK cells express first CD94, then NKG2A, NKG2E, and finally NKG2C. This sequence suggests an immature CD94- and a mature CD94+ subtype of NTENL. Using a rKIR-R without a mTCR-R as a criterion in 25 cases of NTENL, we confirmed a true NK lineage in 19 cases, including 10 CD94+ and 9 CD94- patients by reverse transcriptase-polymerase chain reaction (RT-PCR). Eight of the 10 CD94+ patients but only 2 of the 9 CD94- patients survived beyond 1 year (median survival, 60 months versus 10 months by Meier-Kaplan survival analysis, P =.026 by Cox F test). The remaining 6 patients had a rKIR-R plus a mTCR-R, suggesting mixed NK/T differentiation. They were CD94- by RT-PCR, found predominantly in young women, and had a median survival of 35 months. Thus, on the basis of the transcripts of NKRs, a division of NTENLs into CD94+, CD94-, and mixed NK/T types reflects a true biologic divergence with different clinical behaviors.

Objective: In order to ascertain the lateral operation and medial maxillectomy affect on nasal airway., Method: Report 16 cases of nasal cavity or nasal sinus neoplasm who were therapied by lateral rhinotomy and medial maxillectomy. They were observed by acoustic rhinometer (AR) and CT examination in 1-2.5 years after the surgery., Result: The feeling of nasal obstruct were disappeared in 3 months after the surgery. The nasal airway resistance(NAR) of the ill nasal cavity was markedly decreased after the surgery, and the total NAR were decreasing unmarkably. The mucous proliferation was notable only in the first year after surgery, and especially on remains of the inferior turbinate or extra wall of nasal cavity., Conclusion: Lateral rhinotomy operation and medial maxillectomy have less impact on nasal airway and the functions.

Purpose: Inverted papillomas of the nose and paranasal sinuses are uncommon neoplasms, characterized by their tendency to recur and by their association with malignancy. Over the past 25 years, the standard surgical treatment of inverted papillomas has involved extranasal, en bloc resections because of high recurrence rates with less extensive surgery. The past decade has witnessed an increasing number of reports documenting the successful treatment of inverted papilloma with endoscopic approaches. This investigation examines the role of endoscopic procedures in treating inverted papilloma., Materials and Methods: The present article presents a thorough review of the literature relating to the history, pathology, diagnosis, and treatment of inverted papillomas of the nose and paranasal sinuses. It also provides a systematic review of the pertinent medical literature with pooled statistical analysis of data from 33 studies involving 1,426 patients., Results and Conclusions: The findings suggest that many cases of inverted papilloma can be treated successfully with low recurrence rates and less morbidity through endoscopic surgery, with results comparable to extranasal procedures. Less aggressive approaches to the sinuses and nonendoscopic intranasal procedures, such as the Caldwell-Luc, have unacceptable rates of recurrence and should be abandoned. A staging system for inverted papilloma and a logical approach to treatment are presented.

Nitric oxide (NO) can play an important role in the regulation of vascular tone and neurotransmission, as well as in non-specific immunoreactions and inflammation in a variety of tissues. Increased quantities of nitric oxide in respired air can be measured during inflammatory processes. However, the exact role and precise sources of NO under physiological and pathophysiological conditions within the airways remain to be defined. Three isoforms of NO-synthases can be distinguished: two constitutive (neuronal and endothelial) Ca(2+)-dependent cNOS and one inducible Ca(2+)-independent iNOS (NOS II). Constitutive NOS (NOS I and III) release a basal amount of NO under physiological conditions. The inducible form once expressed can catalyse the generation of large quantities of NO. Many kinds of cells, such as macrophages, neutrophils, endothelium and smooth muscle cells, are capable of expressing NOS II. Since all isoforms of NO-synthase seem to be present in nasal tissues and the expression of iNOS under inflammatory conditions seems to be responsible for excessive production of NO, the distribution of NOS-isoforms (especially NOS II) in normal and inflammatory nasal tissue, as well as the exact requirements for expression of iNOS remain to be proven. Non-inflamed fresh human nasal mucosa from the middle turbinate was compared immuno-histologically with nasal mucosa having the typical findings of chronic polypoid rhinosinusitis (i.e., polypoid middle turbinates and polyps of the middle nasal duct). In order to gain more information about the mechanisms of acute inflammation, non-inflamed vital turbinates were incubated in vitro with the proinflammatory substances bacterial lipopolysaccharides (LPS) and tumor necrosis-factor (TNF) for 30, 60, 90, 120, 180 and 240 min. Subsequent to exposure to NADPH-diaphorase and immunostaining with specific antibodies to each NOS-isoform, clearly increased or initiated expressions of inducible NOS (iNOS) in blood vessels, glands, macrophages and epithelium of chronically inflamed and LPS-incubated nasal tissue became apparent in comparison to the non-inflamed controls. In contrast, NOS III/NOS I seemed to be not affected. The onset of immunohistochemically recognizable NOS II expression was observed after 90 min incubation with of LPS/TNF-alpha. Polypoid tissue showed a strong increase in submucosal thickness and a high infiltration of iNOS-positive leukocytes (granulocytes and macrophages) compared to the LPS-incubated non-inflamed specimens. These findings implicate NOS II generated nitric oxide as a key agent for causing swelling, secretion and obstruction in patients with acute and chronic polypoid or allergic rhinitis. These findings also suggest that molecular NO has to be considered in the pathophysiology of chronic polypoid rhinosinusitis.

In this in vivo prospective, controlled study, we have examined the capsaicin-induced levels and secretion patterns of the colocalized neuropeptides substance P, calcitonin gene-related peptide (CGRP), and neurokinin A in nasal secretions of subjects with nasal polyps, and we compared these with secretion patterns from healthy subjects and from subjects with allergic rhinitis. Capsaicin was used to elicit neuropeptide release. The neuropeptide levels were measured by an ELISA technique. For substance P, subjects with nasal polyps responded very poorly to capsaicin stimulation. The atopic group was more reactive to capsaicin stimulation than control subjects. For CGRP the increase was immediate in all groups. Atopic subjects and subjects with polyps had a less pronounced but sustained response to capsaicin stimulation. CGRP levels in atopic subjects and those with polyps were restored rapidly. Atopic subjects had higher neurokinin A levels with an immediate and sustained response to capsaicin. Control subjects had higher levels than those with polyps, but both groups were nonresponsive to capsaicin stimulation.

Since 1992, 42 patients at the University of Pennsylvania have been treated for inverted papilloma (IP). Thirty-three patients were managed endoscopically with or without a Caldwell Luc approach (CLA) and retrospectively analyzed. The CLA, which involves a gingivobuccal incision for access to the maxillary sinus, is distinguished from a formal Caldwell Luc procedure. These 33 patients with histologically confirmed IP were without evidence of malignancy. They also did not have evidence of intracranial, orbital, or frontal sinus IP. Seventeen of 33 patients (17/33) were without prior treatment (primary). Sixteen of 33 (16/33) presented from an outside institution with recurrent IP (secondary). The recurrence rate using this method to treat primary IP was 6% (1/17), and for secondary IP was 25% (4/16). Scheduled postoperative endoscopic surveillance permitted early detection of recurrence and continued endoscopic control of IP. All 33 patients were endoscopically free of disease at the end of the study. These preliminary data are encouraging for the use of intranasal endoscopy with and without CLA as a means of managing and controlling IP in selected cases.

Objective: To characterize the relationship between allergic rhinitis, the severity and duration of nasal disease, olfactory function, and self-reported olfactory symptoms, including fluctuations or distortions in odor perception., Design: Assessment of olfactory function and symptoms of 90 patients with allergic rhinitis., Setting: A clinic of a university teaching hospital and research facility., Patients: Sixty patients who presented to the Taste and Smell Clinic who had positive allergy test results and 30 patients who presented to the Allergy-Immunology Clinic. The Taste and Smell Clinic patients were grouped by nasal-sinus disease status (30 without chronic rhinosinusitis or nasal polyps, 14 with chronic rhinosinusitis but without polyps, and 16 with nasal polyps)., Main Outcome Measures: Subjective olfactory symptom questionnaire and objective olfactory function tests., Results: The Allergy-Immunology Clinic patients were diagnosed as being normosmic and the Taste and Smell Clinic patients as being hyposmic or anosmic with olfactory loss that increased significantly with nasal-sinus disease severity. Comparisons with normative data confirm that olfactory scores observed in all groups were significantly lower than expected because of the aging process alone. The self-reported duration of olfactory loss increased significantly with nasal-sinus disease severity. The Taste and Smell Clinic patients without chronic rhinosinusitis or nasal polyps reported the greatest incidence of olfactory distortions and olfactory loss associated with upper respiratory tract infections., Conclusions: There appears to be a continuum of duration and severity of olfactory loss in allergic rhinitis that parallels increasing severity of nasal-sinus disease. As a result of the increased frequency of respiratory infection associated with allergic rhinitis, these patients are at risk for damage to the olfactory epithelium.

Cystic fibrosis (CF) is the most commonly inherited disease in Caucasians and is caused by a mutation in the gene encoding a membrane transport protein. This cystic fibrosis transmembrane conductance regulator (CFTR) is thought to be an apical Cl- channel activated by intracellular cAMP. Most recent findings suggest that CFTR is more than a pure Cl- channel and might be involved in the regulation of other transport systems. In the present study we show that CFTR as a Cl- channel plays only a minor role in primary cultured human nasal epithelium derived from non-CF and CF patients. These findings are especially of interest for non-CF human nasal epithelia in which CFTR is correctly inserted. In both tissues Cl- secretion is negligible as compared with Na+ absorption. We confirm and expand our previous observations that Na+ absorption in human nasal epithelium is the dominant ion transport process and that Cl- secretion is detectable in both CF and non-CF tissue. Moreover, we show that cAMP and ATP were not able to stimulate any silent Cl- channels in CF or non-CF human nasal epithelial cells. We further give evidence that in human nasal CF and non-CF epithelium Na+ absorption is mediated by epithelial Na+ channels (ENaC) that are either different from those of other epithelia or which exhibit altered regulation. These differences between Na+ channels of human nasal epithelium and "classical" epithelial Na+ channels include lack of activation by the intracellular second messenger cAMP and the steroid hormone aldosterone. We show further that human nasal Na+ channels are inhibited by Cl- channel blockers and exhibit a different pharmacology towards common Na+ channel blockers.

We describe 8 patients affected with Costello syndrome including an affected sib pair and review the literature on 29 previously reported cases. We emphasize an association with advanced parental age, which is consistent with autosomal dominant inheritance with germline mosaicism. The pathogenesis appears to involve metabolic dysfunction, with growth disturbance, storage disorder appearance, acanthosis nigricans, hypertrophic cardiomyopathy, and occasional abnormalities of glucose metabolism. Although the cause is currently unknown, Costello syndrome is interesting because of a potential genetic-metabolic etiology.