Primary Cervical Extraosseous Ewing's Sarcoma Originated from the Sternocleidomastoid Muscle: A Case Report and Review of the Literature.

Extraosseous Ewing's sarcoma is extremely rare in the soft tissues of the neck, especially in the sternocleidomastoid muscle. It usually manifests clinically as a rapidly growing mass that shows great potential for local spread. The aim of this paper is to present a rare case of еxtraosseous Ewing's sarcoma in the sternocleidomastoid muscle. To the best of our knowledge, this is the first case of extraskeletal Ewing's sarcoma at this location. The patient was admitted to our clinic because of a neck tumefaction. The computerized tomography finding showed a tumor mass, most of which was in the V region of the neck, measuring 40 × 27 × 35 mm. Pathohistological and immunohistochemical findings showed that it was Ewing's sarcoma. Unfortunately, the patient passed away nine months after the initial diagnosis. Extraosseous Ewing sarcoma is a rare, fast-growing malignant tumor manifesting histomorphological similarities to bone Ewing's sarcoma. Most reports state that extraosseous Ewing sarcoma has a worse prognosis than skeletal. Extraosseous Ewing sarcoma should be borne in mind in the differential diagnosis of soft tissue tumors of the neck.

The Impact of Cytokines on Health-Related Quality of Life in Adolescents with Allergic Rhinitis.

BACKGROUND: Frequent episodes of nasal symptoms are the usual clinical manifestations (CM) of allergic rhinitis (AR) and have a significant negative impact on health-related quality of life (HRQoL) in adolescents. The purpose of this cross-sectional study was to test the hypothesis that cytokines in nasal mucus may be associated with HRQoL in adolescents with AR. METHODS: European Quality of Life 5 Dimensions 3 Level Version (EQ-5D-3L), "The Adolescent Rhinoconjunctivitis Quality of Life Questionnaire" (AdolRQLQ) and the Total 4 Symptom Score (T4SS) scoring system were administered to 113 adolescents with AR, nonallergic rhinitis (NAR) and to healthy control subjects. Nasal secretions were sampled and tested for 13 cytokines using a multiplex flow cytometric bead assay. RESULTS: The AR group had significantly lower EQ-5D-3L (0.661 ± 0.267 vs. 0.943 ± 0.088; p < 0.001) and higher AdolRQLQ total scores (2.76 ± 1.01 vs. 1.02 ± 0.10; p < 0.001) compared to the control group. The AR group had higher concentrations of IL-1ß (p = 0.002), IL-6 (p = 0.031), IL-8 (p < 0.001), IL17-A (p = 0.013) and IL-18 (p = 0.014) compared to the control group, and IL-1ß, IL-6, IL17-A and IL-18 were significantly (p < 0.050) increased with disease progression. Cytokines IL-1ß, IL-6, as well as severe CM, were identified as significant predictors of lower HRQoL in adolescents with AR. CONCLUSIONS: This study identified IL-1ß, IL-6, as well as severe CM, as predictors of lower HRQoL in adolescents with AR. However, these results should only serve as a starting point for additional confirmation research.

Clinical Characteristics and Treatment Experiences of Pediatric Acute Mastoiditis and Its Complications at the University Tertiary Care Center in the 10-Year Prevaccinal Period.

Introduction: We aim to provide an overview of the clinical characteristics and treatment of pediatric acute mastoiditis (AM) and its complications in the prevaccinal pneumococcal period. Materials and methods: Retrospective case series. An analysis of pediatric patients with AM treated at a university tertiary care center from 2008 to 2018 was performed. Results: The research included 121 children, and 27.3% of them had some form of complication. The mean age at presentation of AM was 3.7 years (range = 0-18 years). The most common extracranial complication of AM was a subperiosteal abscess (n = 25, 75.8%) and the most common intracranial complication was meningoencephalitis (n = 2, 6%). The most common pathogen isolated in the complicated AM was Streptococcus pneumoniae (n = 17, 51.5%). A total of 60% of patients reported antibiotic use before hospital admission, mostly third-generation cephalosporins (37.5%). There was a statistically significant difference between age group and occurrence of complications (P = .001). Females had complications more frequently than males (P = .035). There were no statistically significant differences in levels of inflammatory parameters (C-reactive protein and leukocyte count) between patients with or without complications (P = .373 and P = .124; respectively). All patients with complications of AM were surgically treated. Mortality was 0% and all children completely recovered. Conclusion: Extracranial and intracranial complications of AM required surgical treatment and extended antibiotic therapy. Inflammation parameters did not have a predictive role in identifying children with complications of AM. Further investigations will determine whether the introduction of the mandatory pneumococcal vaccine in our country has led to a reduction in the incidence of AM and its complications.

Gains in Quality of Life in Chronic Otitis Media Patients After Surgery, Adjusted for Placebo Bias.

OBJECTIVE: Using a short-form specific questionnaire, we sought to quantify specific and generic benefits of surgery for chronic otitis media (COM) while adjusting for expectancy (placebo) bias. STUDY DESIGN: A prospective observational pretreatment/posttreatment study. SETTING: A national tertiary referral center. PATIENTS: Consecutive adult COM patients were enrolled for 1 year. Of 246 patients, 205 were recommended for surgery, and 167 were operated on the (more) affected ear. INTERVENTIONS: All patients filled out two questionnaires, one specific (Chronic Otitis Media Questionaire-12) and one generic (Short Form-36) at baseline, and then again 6 and 12 months after surgery. MAIN OUTCOME MEASURES: Factor-based scores of questionnaires, standardized response means (SRMs) for treatment effects, with multiple linear regression for implementing bias adjustment. RESULTS: All but one (generic Short Form-36 "mental" scale) of the seven considered measures gave very highly significant ( p < 0.001) improvements. Unadjusted SRMs were large (1.0-2.0 standard deviation units) for audiometry, symptoms of ear discharge, reported hearing, and aggregate specific quality of life, but only moderate for the less specific activity/healthcare. The proposed bias adjustment reduced SRM magnitudes for most measures by about a third, for activity/healthcare and ear discharge by only a tenth to a fifth, and for audiometry not at all. CONCLUSION: Most scores of the specific questionnaire displayed definite placebo-like biases; this demands caution in interpreting improvement after COM surgery. With bias adjustments, credible and worthwhile magnitudes of improvements remained for Chronic Otitis Media Questionnaire-12 total and subscores (0.5-1.09 standard deviation SRM), but not for generic quality of life.

Syringocystadenoma Papilliferum in the External Ear Canal.

Out of all benign tumors of the ceruminous glands, syringocystadenoma papilliferum is the rarest and represents only 2% of cases. It is an extremely rare benign tumor that originates from modified apocrine sweat glands. The aim of this paper was to present, according to our findings, the 18th case of syringocystadenoma papilliferum in the external auditory canal, with a detailed review of its clinical, radiological and histomorphological characteristics. A 59-year-old man reported to our clinic due to a 5×5 mm papillomatous growth at the entrance to the right external auditory canal. Histopathology indicated, after an excisional biopsy, that it was a syringocystadenoma papilliferum. The resection lines were free of tumor tissue, and the patient has no signs of tumor recurrence. Although rare, it should be considered as a differential diagnosis of lesions in this region. Complete excision is mandatory in order to avoid recurrence and potential malignant alteration.

Is otitis media with effusion associated with Samter's triad a new nosological entity? A preliminary report on inflammatory mediator production.

PURPOSE: Otitis media with effusion (OME) associated with Samter's triad (ST) is a difficult entity to treat. The aim of study was an investigation of the middle ear and nasal production of inflammatory mediators (IM) in patients with ST and analysing differences between them and controls. METHODS: Prospective case-control study. Nineteen patients with OME (five had allergic rhinitis, four had nasopharyngeal lymphoid hyperplasia, five had no evident sino-nasopharyngeal disease and five had confirmed ST) and 15 healthy participants were included. The concentrations of IM interleukin-1 beta (IL-1ß), interferon-alpha 2 (IFN-α2), interferon-gamma (IFN-γ), tumor necrosis factor-alpha (TNF-α), monocyte chemoattractant protein-1 (MCP-1), IL-6, IL-8, IL-10, IL-12p70, IL-17A, IL-18, IL-23 and IL-33 were measured in nasal and middle ear secretions. RESULTS: There was a difference that was close to a level of statistical significance only for IL-1ß levels in middle ear fluid (p = 0.052) between the ST subgroup and the other patients with OME. Also, we found a significant difference for IL-23 in nasal secretions between these subgroups (p = 0.040), whereas the difference in nasal fluid IL-33 was close to a level of statistical significance (p = 0.052). There was a significant difference in nasal concentrations of IL-1ß, IFN-α2, MCP-1, IL-8, IL-18 and IL-33 (p < 0.001, p = 0.005, p = 0.008, p = 0.011, p = 0.011 and p = 0.011, respectively) between the OME group and the healthy subjects. There were significant positive correlations between concentrations of IL-1ß, IFN-α2, IFN-γ, TNF-α, MCP-1, IL-17A, IL-18 and IL-33 (p < 0.001, p < 0.001, p = 0.002, p = 0.028, p < 0.001, p < 0.001, p < 0.001 and p < 0.001, respectively) in nasal and middle ear secretions. CONCLUSION: This preliminary report showed some differences in IM production between the patients with OME associated with ST and those without it. Our results suggest a uniformity of the production of nasal and middle ear IM and supported the concept of a united airway respiratory disease.

Malignant Otitis Externa: Causes for Various Treatment Responses.

OBJECTIVES: Malignant (necrotizing) otitis externa (MOE) is an aggressive form of skin inflammation of the external ear with a tendency to spread infection to the temporal bone. The study aimed to evaluate a causal relationship between treatment responses and clinical features in patients with MOE. MATERIALS AND METHODS: In a retrospective, descriptive section study, the database was analyzed between January 2008 and December 2018 in our department, all patients with diagnosed MOE were identified. RESULTS: A total of 30 patients were evaluated, of which 27 men and 3 women. The youngest patient was 52 years old while he was eldest 88 years, (mean-71 years old). As the most common comorbidity, diabetes mellitus was found in 23 (76%) subjects. Median duration of symptoms was about 3 months. The most common isolated pathogen was Pseudomonas aeruginosa (47%). Patients with facial nerve palsy and erosion of temporal bone find on computerized tomography affect prolonged stationary treatment (Mean, SD 29.2±8.5 and 26,7±11.6 days), while 80% of patients with facial nerve palsy had recurrence of disease (p=0.005) with mean duration of clinical remission of 60±17.3 days. Overall length of treatment is also increased in the presence of comorbidities as well as in patients with cranial nerve involvement. CONCLUSION: Patients with cranial nerve involvement, erosion of temporal bone and presence of comorbidities affect prolonged treatment and adverse prognosis. Early diagnosis and initiation of aggressive therapy are essential for stopping the further spread of the disease and prevention of serious complications.

Chondroma of the Tongue.

INTRODUCTION: Chondromas are rare mesenchymal benign tumors that are most often encountered in pelvis and ribs, and rarely in head and neck region. They account for about 10% of all benign chondroid proliferations and the rarest occurrence of soft tissue chondromas is within oral cavity. CASE: A 10-year-old female patient was referred to our Clinic because of moderate speech impairment, swallowing disturbances and foreign body sensation in tongue. On clinical examination and MRI scans, hard, painless, clearly demarcated submucosal mass was observed on the left side of the tongue. Surgery was perfomed using intraoral incision approach, and the tumor was completly removed. Histopathological examination of the specimen was positive for chondroma. CONCLUSION: Chondromas benign tumors of mature hyaline cartillage localized in soft tissues of head and neck region. Growing slowly, and painlessly, they remain silent for some period, until reaching certain dimensions, and becoming symptomatous. Magnetic resonance imaging scan provides complete information about localization, size, growth of the tissue, and contributes significantly to the decision of appropriate surgical approach.

OTOLOGICAL MANIFESTATIONS OF TURNER SYNDROME: CLINICAL AND RADIOLOGICAL FINDINGS.

INTRODUCTION: Turner syndrome is a chromosomal abnormality where all or a part of one of the X chromosomes is absent or it has other abnormalities. Besides characteristic abnormalities of short stature and infertility, women with Turner syndrome have increased risks for tumors of the central nervous system, especially meningioma and an otologic disease. Meningioma involving the middle ear is extremely rare, and this condition has never been published in association with Turner syndrome. CASE REPORT: We present an otologic manifestation associated with other abnormalities in a patient with Turner syndrome and discuss diagnosis and possible treatment options. CONCLUSION: Multidisciplinary team approach is essential in these patients in order to evaluate their vulnerability and define therapeutic priorities.

Giant destructive sinonasal polyposis.

INTRODUCTION: Authors report their clinical experience in managing a 46-year-old male patient with long lasting nose breathing difficulties caused by nasal obstruction due to a large bilateral tumor masses in both nasal cavities. CASE OUTLINE: Physical examination, laboratory and biochemistry analyses, as well as computed tomography showed an inhomogeneous soft-tissue tumor mass completely filling both nasal cavities, maxillary, ethmoidal, sphenoidal, and frontal sinuses on both sides, accompanied by destruction of bony walls of all sinuses. Preoperative histopathology analysis showed a polyp with squamous metaplasia.The gigantic polypoid mass was removed by bicoronal approach to the frontal and ethmoidal sinuses and by direct approach to the maxillary sinuses and nasal cavity. Definite histopathology analysis confirmed the initial diagnosis, but the presence of fungal hyphae in allergic mucus was also observed. CONCLUSION: Polypoid growth in the nose rarely grow to such gigantic dimensions that it causes destruction of all walls of paranasal sinuses. Considering so far published reports from the literature, the presented case is among the biggest nasal polyps reported until now.

Clinical outcomes and quality of life in patients with nasal polyposis after functional endoscopic sinus surgery.

The majority of studies have shown that the use of functional endoscopic sinus surgery (FESS) leads to symptomatic improvement in 73-98.4 % of patients with chronic rhinosinusitis and nasal polyposis (NP). The aim of the study is to evaluate clinical outcomes and quality of life (QoL) in patients with NP after FESS. The prospective study included 85 consecutive adult patients (≥18 years) with NP who were operated on using FESS after failure of the medicamentous treatment and in certain cases of surgical treatment. QoL was assessed by Short Form-36 Health Survey (SF-36) questionnaire, and the symptom intensity was presented using visual analogue scale (VAS). The objective finding was presented as endoscopic and computerized tomography (CT) score. The intensity of each symptom, the values of symptom scores (major, minor and total), the values of dimension scales and summary scales of the QoL, as well as the values of endoscopic score through three periods of time (pre-surgery, 6 and 12 months after the surgery) were analyzed. Following the FESS, mean intensity values of all individual symptoms and symptom scores were significantly lower and the values of all dimension scales and summary scales of QoL were significantly higher (p < 0.05). There was no statistically significant difference in symptom intensity and QoL after 6 and 12 months of surgical treatment (p > 0.05). Endoscopic score was on average significantly lower after 6 and 12 months of FESS (p < 0.05), but the mean score value after 12 months of operation was significantly higher in relation to that after 6 months of surgery (p < 0.05). Nevertheless, the recurrence of NP was observed in 28 patients (32.9 %) in the follow-up period. In conclusion, FESS in NP patients results in significant improvement of symptom intensity, QoL and endoscopic score. While the intensity of symptoms and QoL showed a tendency to maintain between 6 and 12 months after surgery, endoscopic score showed a tendency of exacerbation in the same period.

Influence of asthma on quality of life and clinical characteristics of patients with nasal polyposis.

Recent studies have evidenced that nasal polyposis (NP) may lead to significant limitations in physical, emotional and social aspects of life of the affected patients. The study is aimed to investigate the influence of asthma on quality of life (QoL), intensity of symptoms, endoscopic and computerized tomography (CT) sinus findings in patients with NP. The cross-sectional study included 88 adult patients with NP out of whom 35 (39.8 %) were asthmatic while 53 (60.2 %) were non-asthmatic. QoL is assessed based on Short Form-36 Health Survey (SF-36) questionnaire, while symptom intensity was presented using visual analogue scale (VAS). The objective finding is presented as endoscopic and CT score. Comparison of individual symptom intensity, total score and major symptom score failed to evidence any statistically significant difference between the groups. Minor symptom score which include intensity of headache, fetor ex ore, fatigue/malaise, dental pain, cough, pressure/fullness in the ears and fever was higher in the group with asthma (p < 0.05). Comparison of scores according to SF-36 domains, as well as summary scores for physical and mental health did not reveal statistically significant difference between the observed groups. Mean value of the endoscopic score in the group with asthma was 8.57 ± 2.22, being 8.38 ± 1.93 in the group without asthma (p > 0.05). Mean value of the CT score in the groups with and without asthma was 20.37 ± 4.34 and 17.47 ± 4.75, respectively (p < 0.01). Asthma has no influence on QoL and endoscopic findings of patients with NP, however it influences minor symptom score and CT findings.

[T lymphocyte function in laryngectomized patients].

AIM: Malignant tumors of the larynx account for 2.3% of all malignancies, while their frequency among tumors of the head and neck ranges between 12% and 20%. Research on the general immune competence in patients with malignant diseases has provided useful insight in the relationship between immune disorders on one side and the clinical course on the other. Unfortunately, only few complete studies have been published so far with this regard in patients with malignant tumors of the larynx, and therefore our study was essentially aimed at establishing of general immunocompetence, presence and levels of the possible immune disorders and their association with the malignant tumors. MATERIAL AND METHOD: The study included forty two patients with primary squamocellular laryngeal cancer. All the patients underwent surgery, out of whom fifteen were treated postoperatively with radiotherapy. We tested the immune competence prior to the operation and in the postoperative period nine months later. In the venous blood we examined T lymphocyte function, monocyte levels and mononuclear phagocyte function. RESULTS: Preoperative evaluation of the presence and levels of general immune competence in patients with laryngeal cancer, showed a distinct decrease in the proliferative response to the PHA mitogen in vitro, with a tendency to normalize in patients who do not develop a relapse of the disease or distant metastasis during the follow-up period. During the whole study period, the number of monocytes and mononuclear phagocyte activity was above the normal level. CONCLUSION: The patients with operable laryngeal carcinoma had considerable immune disorders at various levels, primarily at the level of T lymphocytes. Of all the disorders, reduced mitotic activity of T lymphocytes in response to mitogens showed the highest dependance on the presence of malignant tissue in the organism.

[Impact of rhinosinusal polyposis on CT score in patients with chronic rhinosinustis].

BACKGROUND/AIM: Chronic sinusitis is a disease characterized with mucosal inflammation of nasal and paranasal sinuses for at least 12 weeks. In order to assess the extent and severity of inflammatory changes in paranasal sinuses CT score according to Lund-Mackay is the most commonly used. Recent studies show the possibility of existing different subtipes of chronic rhinosinusitis, pointing out the presence of nasal polyps and their influence on the severity of chronic rhinosinusitis. The aim of this research was to examine the influence of sinonasal polyposis on the extensity of inflammatory changes on computerized tomography (CT), evaluated by the Lund-Mackay CT score. METHODS: A prospective study compared the Lund-Mackay CT score values between the patients with chronic rhinosinusitis associated with nasal polyps and those without them. We determined mean values of the total CT score in both groups of the patients, as well as mean values of CT score for each group of sinuses and ostiomeatal complexes. RESULTS: The study included 90 patients, 47 males and 43 females, 45-year old on average, diagnosed with chronic rhinosinusitis on the basis of diagnostic algorithm. The group with uncomplicated chronic rhinosinusitis (without nasal polyps) consisted of 30 patients and the group with complicated chronic rhinosinusitis (with nasal polyps) of 60 patients. Observing these two groups of patients revealed statistically highly important intergroup difference in CT score for each group of sinuses and ostiomeatal complexes. The mean value of total CT score in the group with uncomplicated chronic rhinosinusitis was 4.37 while in the group with complicated chronic rhinosinusitis it was 16.05 (p < 0.01). CONCLUSION: Chronic rhinosinusitis complicated by sinonasal polyposis is characterized with more extensive inflammatory changes on CT and, consequently, with higher CT score for each group of sinuses and ostiomeatal complexes, as well as higher total CT score.

Middle ear tuberculosis: diagnostic criteria.

INTRODUCTION: Tuberculous otitis is a diagnostic problem due to the difficulty to obtain microbiological, histomorphological and cytological confirmation of the disease. OBJECTIVE: Our objective was to compare clinical and radiological characteristic and development of otogenic complications in patients with tuberculous otitis and otitis with cholesteatoma as the most destructive form of chronic nonspecific otitis in the purpose of establishing the diagnostic criteria for tuberculous otitis. METHODS: Medical records of 12 patients with tuberculous otitis and 163 patients with cholesteatoma treated at the Institute of Otorhinolaryngology and Maxillofacial Surgery in Belgrade during the eight-year period were analyzed. All of the patients underwent otomicroscopic, audiological and radiological examination of the thorax and temporal bone, microbiological examination of the secretion and histomorphological examination of the tissue taken during middle ear surgery. Statistical analysis was done using chi2 test with Yates correction. RESULTS: Otogenic complication as facial palsy and sensorineural hearing loss were more frequent in tuberculous otitis patients, than in cholesteatoma. Also, fistulas of the labyrinth and facial canal bone destruction were also more frequent in tuberculous otitis than in cholesteatoma. A larger extent of temporal bone destruction was noticed on CT scans of the temporal bone in half of the patents with tuberculous otitis. Coexistence with miliary pulmonary tuberculosis was detected in one third of the patients. There were no microbiological or histomorphological confirmations of the disease, except in one case with positive ZiehI-Neelsen staining. CONCLUSION: Tuberculous otitis media should be considered in patients with serious otogenic complications and with shorter duration of ear discharge, and in association with diagnosed miliary pulmonary tuberculosis and extensive temporal bone destruction. Polymerase chain reaction still is not reliable for diagnosis.

Comparison of effects of endogenous and exogenous excitatory amino acids on Retzius nerve cells of the leech.

In this paper we examine the effects of L-aspartate, L-glutamate, and beta-N-oxalylamino-L-alanine (Lathyrus toxin) on Retzius nerve cells of the leech Haemopis sanguisuga. The goal was to compare the electrophysiological effects of endogenous vs. exogenous amino acids, known as potent neurotoxins, through the mechanism ofexcitotoxicity. We used classical intracellular recordings on Retzius nerve cells in isolated ganglia of the leech, and plotted dose-response curves to compare potencies. Our results show that Lathyrus toxin is more than 200 times more potent in depolarizing the membrane potential on our model than L-aspartate and L-glutamate, which are approximately equipotent.

The analysis of clinical characteristics of the chronic rhinosinusitis: complicated and uncomplicated form.

The objective of our study was to analyze the intensity of subjective symptoms and objective findings of endoscopy and CT scanning in chronic rhinosinusitis, in the groups with and without nasal polyps. To evaluate the intensity of subjective symptoms visual analogue scale (VAS) was used, while scores were obtained by adding grades. Endoscopic finding was given in scores recommended by Lanza and Kennedy and CT results were presented by Lund-Mackay scoring system. The study included 90 consecutive adult patients, 47 males (52%) and 43 females (48%), mean age 45 years. The group with chronic rhinosinusitis without nasal polyps (uncomplicated form) consisted of 30 patients, while the group with polyps (complicated form) included 60 patients. Comparing mean intensity values of all subjective symptoms between these two groups we found out that nasal obstruction, nasal secretion and hyposmia were significantly more manifested in the polyp group (P<0.01). Facial congestion was also more manifested in the polyp group (P<0.05). Mean score value of major symptoms was 35.55 in the polyp group, and 23.13 in the group without polyps (P<0.01). Mean value of total symptom scores was 48.68 in the polyp group, and 35.00 in the group without polyps (P<0.01). Endoscopic score was approximately 9.03 in the polyp group, and 2.43 in the group without polyps (P<0.01). CT score was 16.05 on an average in the polyp group, and 4.37 in the group without polyps (P<0.01). Chronic rhinosinusitis complicated by nasal polyposis is characterized by higher degree of nasal obstruction, nasal secretion, hyposmia and facial congestion, which results in higher score of major and total score of symptoms, respectively. This form is also characterized by worse objective findings, which is reflected in higher endoscopic and CT scores.