Nasopharyngeal angiofibroma in an adult with Proteus syndrome. First reported case.

BACKGROUND: Proteus Syndrome (PS) is a rare hamartoneoplastic disorder consisting of a disproportionate and asymmetric overgrowth of body parts, leading to severe body and face disfigurement. Individuals with PS frequently need to undergo a number of surgical procedures including Otolaryngology interventions. Their peculiar anatomy turns even the most straightforward intervention to a challenging one. CASE DESCRIPTION: A 31-year-old adult male with PS presented with recurrent epistaxis and nasal obstruction. A nasal mass was found during the physical examination, which was surgically removed under general anesthesia. Histology revealed juvenile angiofibroma. CONCLUSION: This is the first reported case of a patient with PS presenting with juvenile nasopharyngeal angiofibroma. Although vascular malformations may appear in these patients, juvenile nasopharyngeal angiofibroma should be taken under consideration when PS patients present with recurrent epistaxis. HIPPOKRATIA 2017, 21(3): 147-149.

Expression of type III interferons (IFNλs) and their receptor in Sjögren's syndrome.

Type III interferons (IFNs) or IFN-λs (IFN-λ1/IL29, IFN-λ2/interleukin (IL)-28A and IFN-λ3/IL-28B) consist of a recently identified group of IFNs, implicated initially in several human diseases, including cancer and autoimmunity. In this study, we sought to investigate the expression of type III IFNs and their common receptor IFN-λR1/IL-28Ra in Sjögren's syndrome (SS). Type III IFN expression was examined in minor salivary gland tissues (MSG), peripheral blood mononuclear cells (PBMCs), sera and resting or Toll-like receptor (TLR)-stimulated salivary gland epithelial cells (SGEC) from SS patients and sicca-complaining controls. All type III IFN family members were detected in ductal and acinar epithelia of MSGs from both SS patients and sicca controls. IFN-λ2/IL-28A and IFN-λ3/IL-28B were also expressed in infiltrating mononuclear cells. In SS patients with intermediate MSG lesions, the epithelial expression of IFN-λ2/IL-28A was more intense compared to sicca controls (P < 0·05). The receptor IFN-λR1/IL-28Ra was detected in all types of cells except fibroblasts, and was exceptionally strong in plasmatocytoid dendritic cells, indicating that they are susceptible to type III IFN-mediated regulation. In the periphery, only IFN-λ1/IL-29 was detected in the sera and was elevated significantly in SS patients with intermediate MSG inflammatory lesions compared to sicca controls (P = 0·0053). None of the type III IFNs was expressed constitutively in resting SGECs; they were all induced readily by TLR-3 stimulation, suggesting that the in-situ epithelial expression can be attributed to local microenvironment. Type III IFNs are expressed in MSGs in a similar pattern to type I IFNs and their expression is probably subjected to micro-environmental regulation, suggesting that they are implicated in the inflammatory processes occurring in the affected exocrine glands.

Adhesion molecules in cancer of the head and neck: role of dysadherin.

Dysadherin is a recently discovered cancer-related cell membrane glycoprotein that has an important role in tumor progression and metastasis. We are focusing on the role of dysadherin in E-cadherin downregulation, the different expression patterns of the molecule in cancer of the head and neck and its potential role as a molecular target for future applications in diagnosis, prognosis, and management of the disease.

Neurotoxicity of vincristine on the medial olivocochlear bundle.

OBJECTIVE: Vincristine is a well known neurotoxic chemotherapeutic agent. Dose dependent and cumulative peripheral neuropathy is the main dose limiting side effect of chemotherapy with vincristine. The mechanisms responsible for the neurotoxic effects of vincristine have not yet been fully understood. This prospective study was directed at determining whether vincristine treatment interferes with the function of the medial olivocochlear bundle. DESIGN: Fifteen children suffering from leukemia were subjected to tympanogram, stapedial muscle reflex, pure tone audiometry and transient evoked otoacoustic emissions (TEOAEs) in the absence and presence of contralateral white noise on day 1 and on day 22 of treatment with vincristine. The function of the medial olivocochlear bundle was assessed by the phenomenon of suppression of otoacoustic emissions by contralateral application of white noise. RESULTS: The study revealed a statistically significant decrease of contralateral suppression amplitudes in all cases after three sessions of chemotherapy with vincristine. On the contrary no alterations were observed in pure tone audiometry thresholds. A non-significant decrease of the mean TEOAEs' amplitudes was also noted. When analyzed by frequency, however, this decrease reached the level of statistical significance at two frequencies. CONCLUSION: Vincristine treatment seems to exert a neurotoxic effect on the efferent olivocochlear system, which takes place early in the course of chemotherapy. This is a new aspect to be added to the possible mechanisms underlying the toxicity of vincristine in the auditory periphery. Whether changes in efferent function might contribute to understanding the mechanisms of neurotoxicity caused by vincristine, or find any clinical application as a predictor or early detector of neurological side effects of vincristine still remains to be seen.

Ethmoidal sinus adenocarcinoma with orbital invasion.

PURPOSE: To report a rare case of massive ethmoidal adenocarcinoma with orbital invasion but minimal ophthalmic symptoms on presentation. METHODS: Case report of a 69-year-old, otherwise healthy, retired carpenter who was referred for treatment of bilateral senile cataract. RESULTS: A relative afferent pupillary defect and sectorial disc atrophy on ophthalmic examination led to further investigation and identification of an extensive ethmoidal neoplasm with orbital invasion. An incisional biopsy was performed and histopathologic examination revealed an adenocarcinoma of low-grade malignancy. CONCLUSION: Ethmoidal adenocarcinomas with orbital involvement may occasionally be relatively asymptomatic and masked by coexisting ocular disease.

A comparison of the efficacy and patient acceptability of budesonide and beclomethasone dipropionate aqueous nasal sprays in patients with perennial rhinitis.

This was an open, randomized, cross-over study comparing the efficacy and acceptability of aqueous nasal suspensions of budesonide, 200 micrograms b.i.d. and beclomethasone dipropionate (BDP), 100 micrograms q.i.d., each given for 6 weeks to patients with perennial rhinitis and a history of allergy. Forty men and women aged 18-65 years with perennial rhinitis diagnosed at least 1 year previously, were recruited for study provided they had at least two of the following symptoms of rhinitis--blocked nose, runny nose, itching nose or sneezing. They were requested to record the presence or absence of nasal and ocular symptoms on a severity scale of 0-3 (none, mild, moderate, severe) in daily diary cards. The sum of the nasal scores was calculated to give the total nasal symptom score. Mean individual symptom scores and total symptom score were calculated for each treatment. Thirty-seven patients completed the study. The mean total nasal symptom score was significantly lower during budesonide (2.13) than during BDP (2.75), P = 0.001. There were significantly fewer reports of blocked nose (P = 0.004), runny nose (P = 0.0005) and sore eyes (P = 0.047) during budesonide treatment compared with BDP. Four patients reported adverse events during budesonide treatment (two had nosebleeds and two nasal dryness) and three patients during BDP treatment (two had nasal dryness and one gastric discomfort). A significantly greater proportion of patients stated a preference for budesonide than for BDP on the basis of effect (P = 0.0001), side-effects (P = 0.01), and overall (P = 0.0001).