The role of Nrf2 in pathology of pleomorphic adenoma in parotid gland.

BACKGROUND: Pleomorphic adenoma (benign mixed tumor) is one of the most common salivary gland tumors. However, the processes involved in its carcinogenesis are not well defined. This study aimed to define the contribution of Nfr2 (nuclear factor (erythroid-derived 2)-like 2) to pleomorphic adenoma pathology. The Nrf2-controlled gene system is one of the most critical cytoprotective mechanisms, providing antioxidant responses. MATERIAL AND METHODS: The study was carried out in pleomorphic adenoma and control parotid gland tissues, investigating gene expression of NFE2L2, as well as KEAP1 (Kelch-like ECH-associated protein 1) and NQO1 (quinone oxidoreductase), at mRNA and protein (immunohistochemistry) levels. Functional evaluation of Nrf2 system in the parotid gland was evaluated in HSY cells (human parotid gland adenocarcinoma cells). RESULTS: Pleomorphic adenoma specimens showed cytoplasmic and nuclear Nfr2 expression in epithelial cells, as well as more variable lower Nrf2 level in mesenchymal cells. In the parotid gland, Nrf2 was expressed in cytoplasm of serous, mucous, and duct cells. Nuclear Nrf2 expression was predominantly seen in serous cells, whereas mucous and duct cells were mostly negative. Comparable mRNA levels of NFE2L2 and NQO1 genes and significantly higher expression of KEAP1 in pleomorphic adenoma were seen. HSY cell incubation with oltipraz demonstrated significant elevation of NFE2L2 after 24 and 48 hours of stimulation, whereas NQO1 was elevated, but significantly only after 24 hours, and KEAP1 expression remained unchanged. CONCLUSIONS: Summarizing both in vitro and in vivo observations, it can be stated that Nrf2 may play a role in the pathology of pleomorphic adenoma.

Selenium as a marker of cancer risk and of selection for control examinations in surveillance.

Publication is summarization of existing data being results of literature review and our experience on usefulness of selenium as a diagnostic marker selection for control examinations in surveillance and as a marker of patients with high risk of cancers.

[Mucoepidermoid cancer of parotid gland in 14-years-old girl--case report]. / Rak sluzowo-naskórkowy slinianki przyusznej u 14-letniej dziewczynki - opis przypadku.

INTRODUCTION: Malignant tumors of salivary glands are rare, especially in children. MATERIAL AND METHOD: We present a case report of 14-years-old girl affected by mucoepidermoid cancer of parotid gland. RESULTS: Patient was admitted to our Department in May 2007 due to small, hard, moveable tumor localized in right mandibular angle. The VII nerve function was normal. Fine needle aspiration revealed inflammatory cells without atypical cytologic features. Intraoperative tumor was hard, with irregular surface, surrounded by capsule, connected with the main trunk of facial nerve and cartilaginous part of external auditory meatus. Tumor was excised together with superficial part of parotid gland and surrounding lymph nodes. After operation no signs of facial nerve damage were observed. Postoperative histopathological examination revealed mucoepidermoid cancer, in 2 out of 6 specimens neoplasmatic cells were seen in border of excised tissue. In June 2007 the second operation was performed. Remain part of parotid gland was removed with right facial nerve and conservative lymph node resection. Facial nerve and its branches were reconstructed with sural and great auricular nerve as donor grafts. The symptoms of nerve damage were observed in early postoperative days adequate to 5th degree of House-Brackman scale (HB5). 4 years after operation function of facial nerve is estimated on HB3. The girl is under regular oncological and laryngological control free of neoplasm recurrence. CONCLUSIONS: Diagnostics and treatment of malignant parotid tumors in children are difficult and clinical picture must be always taken into consideration as the most important factor.

The Tullio effect in a patient qualified for cochlear implantation: Diagnosis, management and rehabilitation performance: A case report.

RATIONALE: A case study on the qualification and treatment of profound hearing loss with a cochlear implant in a patient with a positive Tullio effect is described. To our knowledge this is the first such case reported in the literature. PATIENT CONCERNS: A 46-year-old woman was admitted due to sudden hearing loss in the right ear (RE). The patient had suffered from bilateral hearing loss since childhood and was fitted with hearing aids on the RE successfully, in contrast to the left ear. While undergoing pure-tone audiometry, a positive Tullio effect was observed in the RE. The average hearing threshold for the RE was 95 dB. Due to the lack of effective treatment for sudden hearing loss, the patient was qualified for cochlear implantation. The patient's attempts to place a hearing aid on the RE resulted in dizziness. DIAGNOSES: Computed tomography excluded the presence of a perilymphatic fistula, which could have been the cause of the patient's vertigo. INTERVENTIONS: During the surgical procedure of cochlear implantation, considering the possible mechanisms of the Tullio effect, the incus was removed and the niche of the oval window was filled with fragments of connective tissue. The postoperative course was uneventful. OUTCOMES: Three months after implantation, speech intelligibility in the free field was 80% of the correctly repeated elements of the numerical test, at 65 dB sound pressure level. An acoustic stimulation test was performed during tonal audiometry and no preexisting symptoms were observed. LESSONS: A positive Tullio effect does not contraindicate treating hearing loss by means of cochlear implantation. When the Tullio effect is present, it is necessary to exclude presence of perilymphatic fistula. During cochlear implant surgery, in a patient with a positive Tullio effect, it is reasonable to disconnect the ossicular chain with the simultaneous sealing of the oval window niche.

Survival of Laryngeal Cancer Patients Depending on Zinc Serum Level and Oxidative Stress Genotypes.

Stress contributes to various aspects of malignancy and could influence survival in laryngeal cancer patients. Among antioxidant mechanisms, zinc and the antioxidant enzymes superoxide dismutase 2, catalase and glutathione peroxidase 1 play a major role. The aim of this study was a prospective evaluation of the survival of patients with laryngeal cancer in relation to serum levels of zinc in combination with functional genotype differences of three key antioxidant enzymes. The study group consisted of 300 patients treated surgically for laryngeal cancer. Serum zinc levels and common polymorphisms in SOD2, CAT and GPX1 were analyzed. The risk of death in patients with the lowest zinc levels was increased in comparison with patients with the highest levels. Polymorphisms of antioxidant genes by themselves were not correlated with survival, however, serum zinc level impact on survival was stronger for SOD2 TC/TT and CAT CC variants. GPX1 polymorphisms did not correlate with zinc levels regarding survival. In conclusion, serum zinc concentration appears to be an important prognostic factor for survival of patients diagnosed with laryngeal cancer. When higher zinc levels were correlated with polymorphisms in SOD2 and CAT a further increase in survival was observed.

[Pharyngeal constrictor muscles closure after total laryngectomy is that really beneficial?]. / Czy zszywanie zwieracza gardla dolnego po laryngektomii calkowitej jest korzystne?

UNLABELLED: High pressure of pharyngo-esophageal segment is the most important factor of impaired development of alaryngeal speech (esophageal speech, tracheoesophageal speech) after total laryngectomy. To prevent pharyngo-esophageal spasm in Department of Otolaryngology in Szczecin are used: pharyngoesophageal plastic surgery with interposition of vascular thyroid flap, two-layer (only mucosa) non-muscular pharyngeal closure and tree-layer closure (mucosa and muscle layer leaving inferior pharyngeal constrictor unsutured). AIM OF STUDY: The aim of this study was to compare the pharyngo-esophageal pressure between patients after pharyngo-esophageal plastic surgery and following the non-muscular pharyngeal closure. MATERIAL AND METHODS: One hundred eighty two subjects after total laryngectomy were enrolled in this study, and included 108 patients subjected to the pharyngo-esophageal plastic surgery, 44 patients who underwent the two-layer pharyngeal closure, and 30 patients with the tree-layer closure. To evaluate the pharyngo-esophageal pressures manometric tests were performed, and to asses the pharynx morphology videopharyngoscopy was used. RESULTS: The average pharyngo-esophageal pressure in the group after the pharyngo-esophageal plastic surgery was 32 (min.-5, max. 50) mmHg. After the two-layer non-muscular pharyngeal closure mean pressure was 35 (min.-17, max.-40) mmHg, and after the tree-layer non-muscular pharyngeal closure the average pressure was 22,42 (min. 5, max. 40) mmHg. The average pharyngo-esophageal pressure was significantly lower (p < 0.01) among patients after the tree-layer non-muscular closure. CONCLUSION: The study suggests that the tree-layer non-muscular pharyngeal closure with inferior pharyngeal constrictor unsutured is the preferable method to prevent pharyngo-esophageal spasm after total laryngectomy. However, the efficacy and safety of this surgical procedure should be explored in further multicenter studies.

Prevalence of Human Papillomavirus Infection in the Head and Neck Area of Patients After Kidney Transplantation Treated With Immunosuppressive Therapy.

The introduction of new and stronger immunosuppressive agents has significantly improved the overall survival rate of patients with transplanted organs; however, prolonged use of immunosuppressive agents has led to severe complications, such as the development of de novo malignant cancers. The incidence of malignant tumors is 3 to 5 times greater among renal transplant recipients than that of the overall population. Traditional neoplasms of the head and neck region are associated with the addiction to smoking and drinking alcohol. However, recent studies indicate that cancers of the throat, tongue, and tonsils are primarily associated with the human papillomavirus (HPV) infection. At present, approximately 25% of the head and neck squamous cell carcinomas are associated with HPV infection. Therefore, we aimed to determine the incidence of oropharyngeal HPV infection in recipients with kidney transplants treated with immunosuppressive therapy and to determine the factors that may favor the contraction of infectious diseases. Furthermore, we considered the purpose of vaccination against HPV among transplant recipients. A total of 32 recipients with kidney transplants were included in this study. Medical history was obtained and a throat swab was collected from each patient. The presence of the HPV DNA in the throat was determined using the GP5+/GP6+ primers. According to our results, 28% of the recipients with kidney transplants were positive for oropharyngeal HPV infection. In addition, among the investigated risk factors, early commencement of the sexual life (below the age of 16 years) was significantly correlated with the development of oropharyngeal HPV infection.

Recommendations of the Main Board of the Polish Society of Otorhinolaryngologists, Head and Neck Surgeons for providing services during the COVID-19 pandemic for outpatient and hospital practices.

Recommendations of the Main Board of the Polish Society of Otorhinolaryngologists, Head and Neck Surgeons for providing services during the COVID-19 pandemic constitute the guidance to outpatient and hospital practices in all cases where contact with a patient whose status of COVID-19 is unknown. They have been created based on world publications and recommendations due to the current state of the COVID-19 pandemic. Justification for suspension of planned provision of services in the first phase of a pandemic was presented. The indication of the best medical practices for the time of stabilization, but with the persistence of the risk of COVID-19 infection in the population are discussed. The possibility of providing services in the following months of the pandemic is important. We provide the rationale for launching medical activities and indicate optimal practices until the consolidation of SARS COV-2 prevention and treatment methods.

[Morphology of the glottis and perceptiv-acoustic characteristic of the voice and speech after extended fronto-lateral epiglotttoplasty]. / Morfologia glosni i charakterystyka percepcyjno-akustyczna glosu i mowy po laryngektomii fronto-lateralnej poszerzonej z nastepowa epiglotoplastyka.

UNLABELLED: For optimalisation glottis morphology and its phonatory function after frontolateral laryngectomy (FLL) the reconstruction of larynx is made; in Clinic of Otolaryngology the most common is using epiglottis wit or without its vertical incision. AIM OF THE STUDY: the influence of widening laryngeal resection and epiglottis incision on shape of reconstructed glottis, level and degree of phonatory closure and perceptive--acoustic characteristic of voice and speech. MATERIAL: 39 patients (38 M and 1 F) after FLL widened of false vocal cord (n = 11), false vocal cord and part of cricoid cartilage (n = 22), whole cricoid cartilage (n = 6). Vertical incision of epiglottis was made in 31 cases. Mean age was 52 (min.-39, max.-70) years. METHODICS: videolaryngoscopic examination, subjective voice estimation using GRBAS scale, objective phonetic--acoustic voice analysis. RESULTS: In videolaryngoscopic examination the most common shape of glottis was irregular triangle (n = 24), rhombus (n = 7), half--moon (n = 2), irregular (n = 6). 2 patients were using whisper. In spectrographic recordings (n = 39) only noise character of stimulation source was in 2 patients, noise--periodic with noise component present in whole acoustic spectrum in 37. F0 for single word and sentence vocalized in affirmative and interrogative form had the value of male voices and its changes during speaking were well noted. In subjective and objective estimation, the worse voice quality was after FLL widened of false vocal cord, part or whole cricoid cartilage. CONCLUSION: 1) FLL with subsequent epiglottoplasty in 94% of patients makes satisfactory morphologic and biophysical conditions for production of voice and sociological efficient speech. 2) Widening resection of false vocal cord, part or whole cricoid cartilage has unbeneficial influence on perceptive--acoustic characteristic of voice and speech.

Constitutional CHEK2 mutations are associated with a decreased risk of lung and laryngeal cancers.

Mutations in the CHEK2 gene have been associated with increased risks of breast, prostate and colon cancer. In contrast, a previous report suggests that individuals with the I157T missense variant of the CHEK2 gene might be at decreased risk of lung cancer and upper aero-digestive cancers. To confirm this hypothesis, we genotyped 895 cases of lung cancer, 430 cases of laryngeal cancer and 6391 controls from Poland for four founder alleles in the CHEK2 gene, each of which has been associated with an increased risk of cancer at several sites. The presence of a CHEK2 mutation was protective against both lung cancer [odds ratio (OR) = 0.3; 95% confidence interval (CI) 0.2-0.5; P = 3 x 10(-8)] and laryngeal cancer (OR = 0.6; 95% CI 0.3-0.99; P = 0.05). The basis of the protective effect is unknown, but may relate to the reduced viability of lung cancer cells with a CHEK2 mutation. Lung cancers frequently possess other defects in genes in the DNA damage response pathway (e.g. p53 mutations) and have a high level of genotoxic DNA damage induced by tobacco smoke. We speculate that lung cancer cells with impaired CHEK2 function undergo increased rates of cell death.

Familial association of laryngeal, lung, stomach and early-onset breast cancer.

This study analyzes the incidence of different types of cancer among 2839 first-degree relatives of 760 consecutive, unselected laryngeal cancer patients, compared with the general population. A statistically significant excess was seen for other cancers of the larynx (SIR: 400), lung (SIR: 135) and stomach (SIR: 271), and early-onset breast cancer (SIR: 287). Familial laryngeal cancer may not be a single site-specific cancer syndrome.

Pleomorphic adenoma of salivary glands does not appear to be a BRCA-1-dependent tumour in a Polish cohort.

BACKGROUND: The aim of the study was to examine whether pleomorphic adenoma of salivary glands can occur on the basis of constitutional BRCA-1 mutations. MATERIALS AND METHODS: Two hundred and sixty-eight patients affected by mixed tumour of salivary glands were examined for occurrence of three BRCA-1 mutations dominating in Poland. RESULTS: BRCA-1 mutation was detected in only one of the patients, a female affected by breast cancer and pleomorphic adenoma of parotid gland. Parotid gland tumour showed clinical and histopathological features of typical pleomorphic adenoma with no morphological features of high-grade malignancy, which are characteristic of BRCA-1-dependent tumours. CONCLUSION: Considering the low frequency of BRCA-1 mutation in the examined group and also the absence of features characterizing BRCA-1-dependent tumours in the only BRCA-1-positive case, pleomorphic adenoma of salivary glands should not be recognized as a BRCA-1 dependent tumour.

Correction: Serum selenium levels and the risk of progression of laryngeal cancer.

[This corrects the article DOI: 10.1371/journal.pone.0184873.].

Serum selenium levels and the risk of progression of laryngeal cancer.

BACKGROUND: Observational studies have reported an inverse relationship between selenium status (blood or toenail) and the risk of laryngeal cancer; however, the impact of low serum selenium level on survival has not been evaluated. METHODS: We conducted a prospective study of 296 patients diagnosed with laryngeal cancer in Szczecin, Poland. Serum selenium was measured at diagnosis and prior to treatment. Patients were followed from the date of diagnosis to death at five years. Vital status was obtained by linkage to the Polish National Death Registry. RESULTS: The five-year survival after diagnosis was 82.0% (95% CI: 68% to 91%) for individuals in the highest quartile of serum selenium (> 66.8 µg/L) and was 28.6% (95% CI 19% to 42%) for individuals in the lowest quartile (<50.0 µg/L). In an age- and sex-adjusted analysis, the hazard ratio (HR) for death from all causes was 7.01 (95% CI 3.81 to 12.9) for patients in the lowest quartile of serum selenium, compared to those in the highest quartile. The corresponding multivariate HR was 3.07 (95% CI 1.59 to 5.94). CONCLUSIONS: This study suggests that a selenium level in excess of 70 µg/L is associated with improved outcome among patients undergoing treatment for laryngeal cancer. Further studies are needed to evaluate if selenium supplementation to achieve this level might improve overall prognosis.

[Horizontal glottectomy--oncologic and functional results. Part I. Horizontal glottic laryngectomy--oncologic results and assessment of the protective and respiratory function of the larynx]. / Laryngektomia pozioma glosniowa-- wyniki onkologiczne i funkcjonalne. Czesc I. Laryngektomia pozioma glosniowa--wyniki onkologiczne, ocena funkcji ochronnej i oddechowej.

INTRODUCTION: In the Otolaryngology and Laryngological Oncology Department of the Pomeranian Medical University in Szczecin (Poland) horizontal glottectomy is executed from 1985. MATERIAL AND METHODS: Material consists of 27 patients (26 males and 1 female; an average age 57 min. 40, max.). We evaluated: 1) oncological results, 2) 3 and 5-years survival rate without recurrence of the cancer, 3) evaluation of the protective and respiratory function. Protective function was evaluated basing on: a) period after operation when feeding tube was removed, b) subjective assessment of swallowing liquid and solid food. For evaluating respiratory function we stated period after operation to decannulation and also spirometric investigations were done. RESULTS: From among 27 operated patients died 4 (15%)--one on the second day after operation. Recurrence of the cancer (local or into lymph nodes) were observed in 5/26 (19.2%). Three years without symptoms of recurrence survived 89% (17/19), five years--80% (12/15). Swallowing through natural way was possible during the first 24 hours after operation in 13 of 26 (50%) patients. In the remaining 13 only 2 feeding tubes were removed on the 35th day after operation. Decannulation was possible at 92% (24/26) patients. 15 (58%) patients were decannulated in the first two week after operation. Spirometric investigation (n = 10) showed no restriction and very small obturation on the larynx - very good laryngeal flow nearing to normal. CONCLUSIONS: Oncologic results, protective and respiratory function after horizontal function results are satisfactory and comparable to the other researchers.

[Horizontal glottectomy--oncological and functional results. Part II. Morphology of the glottis and perceptive-acoustics characteristic of the voice and speech after horizontal glottectomy]. / Laryngektomia pozioma glosniowa-- wyniki onkologiczne i funkcjonalne. Czesc II. Morfologia glosni i charakterystyka percepcyjno-akustyczna glosu i mowy po laryngektomii poziomej glosniowej.

INTRODUCTION: The authors showed findings concerning glottis morphology and perceptual-acoustic characteristics of voice and speech after partial classical (PCGLg) and extended glottic partial laryngectomy (PEGLg). MATERIAL AND METHODS: 10 patients (9 M., 1 F. average age 56 (min. 47 max. 65) were examined. All patients were undergone glottic partial laryngectomy: a) classical (n = 5) b) extended of vocal process (n = 3) with (n = 2) or without (n = 1) the removal of the mucous false folds, c) extended of part of arytenoid cartilage with (n = 2) or without (n = 1) the removal of the mucous of the false folds. The following examinations were executed: phoniatric, videolaryngoscopic and perceptual-acoustic analysis. RESULTS: After PCGLg and one extended of vocal process, voice and speech has mostly characterized of features of hypofunction dysphonia. Hyperfunction was found in patients after removal of the mucous of the false folds due to leucoplakia. In case of removing of a part of arythenoid cartilage the notable or entire standstill or lack of full phonatory closure were found. The phonetical-acoustic analysis showed that in patients using melodious voice, the character of the source of actuating was periodically-noise, with the component of noise in all range of the course of the acoustic signal of voice. The parameters such as F0, jitter, shimmer does not make coherent conclusions and are less useful in the assessment of the quality of voice. CONCLUSIONS: In case of the resection of the part of the arythenoid cartilage during glottis laryngectomy, we take into account lack of full phonatory closure and using whisper by the patients. Obtaining the reliable conclusions needs continuations of the investigations and increasing number of patients. These researches are in progress.

[The evolution of epidemiology and clinical characteristics of laryngeal and hypopharyngeal carcinoma in Poland from 1991 to 2001]. / Ewolucja obrazu epidemiologicznego i klinicznego raka krtani i krtaniowej czesci gardla w Polsce w latach 1991-2001.

On the base of retrospective analysis of 12,888 cases of carcinoma of larynx and hypopharynx, diagnosed in 19 ENT Departments in Poland from 1991 to 2001, the assessment of basic epidemiological data, including the localization of tumor and stage of local and clinical advancement of the disease at the time of diagnosis has been conducted. In analyzed period of 11 years the trends to change of the mentioned above parameters has been examined. The significant increase of female patients in this period was observed, with average proportion M:F = 8:1. The glottis localization of carcinoma dominated (47.6%), followed by supraglottis (40.8%) and pyriform fossa (7.8%), with significant increase of pyriform fossa tumors in the analyzed period of 11 years. In the majority of cases the carcinoma of larynx and hypopharynx was diagnosed in the advanced stage (T3 + T4) of local disease, with the highest percentage in localization within the pyriform fossa (81.0%), and the lowest percentage in glottis tumors (45.6%). The regional lymph nodes metastases has been diagnosed in 46.7% of the analyzed group, with the highest percentage in tumors localized in pyriform fossa (82.9%), and the lowest percentage in tumors of glottis localization (33.1%). In the 11 years time the significant drop down of N0 cases and tendency to increase of N2 and N3 in the supraglottis localization of tumor. The distant metastases in the analyzed group at the time of diagnosis has been registered in 2.0%, with the highest percentage in posterior pharyngeal wall (7.6%) and pyriform fossa (7.4%). The authors postulate the renewal of prospective study on epidemiology, clinical characteristics and treatment results of larynx and hypopharynx carcinoma in Poland.

A rare truncating BRCA2 variant and genetic susceptibility to upper aerodigestive tract cancer.

Deleterious BRCA2 genetic variants markedly increase risk of developing breast cancer. A rare truncating BRCA2 genetic variant, rs11571833 (K3326X), has been associated with a 2.5-fold risk of lung squamous cell carcinoma but only a modest 26% increase in breast cancer risk. We analyzed the association between BRCA2 SNP rs11571833 and upper aerodigestive tract (UADT) cancer risk with multivariable unconditional logistic regression adjusted by sex and combinations of study and country for 5942 UADT squamous cell carcinoma case patients and 8086 control patients from nine different studies. All statistical tests were two-sided. rs11571833 was associated with UADT cancers (odds ratio = 2.53, 95% confidence interval = 1.89 to 3.38, P = 3x10(-10)) and was present in European, Latin American, and Indian populations but extremely rare in Japanese populations. The association appeared more apparent in smokers (current or former) compared with never smokers (P het = .026). A robust association between a truncating BRCA2 variant and UADT cancer risk suggests that treatment strategies orientated towards BRCA2 mutations may warrant further investigation in UADT tumors.

The 12p13.33/RAD52 locus and genetic susceptibility to squamous cell cancers of upper aerodigestive tract.

Genetic variants located within the 12p13.33/RAD52 locus have been associated with lung squamous cell carcinoma (LUSC). Here, within 5,947 UADT cancers and 7,789 controls from 9 different studies, we found rs10849605, a common intronic variant in RAD52, to be also associated with upper aerodigestive tract (UADT) squamous cell carcinoma cases (OR = 1.09, 95% CI: 1.04-1.15, p = 6x10(-4)). We additionally identified rs10849605 as a RAD52 cis-eQTL inUADT(p = 1x10(-3)) and LUSC (p = 9x10(-4)) tumours, with the UADT/LUSC risk allele correlated with increased RAD52 expression levels. The 12p13.33 locus, encompassing rs10849605/RAD52, was identified as a significant somatic focal copy number amplification in UADT(n = 374, q-value = 0.075) and LUSC (n = 464, q-value = 0.007) tumors and correlated with higher RAD52 tumor expression levels (p = 6x10(-48) and p = 3x10(-29) in UADT and LUSC, respectively). In combination, these results implicate increased RAD52 expression in both genetic susceptibility and tumorigenesis of UADT and LUSC tumors.

[Embryonal rhabdomyosarcoma of the nasopharynx in a 3-year old girl]. / Rhabdomyosarcoma embryonale nosogardla u 3-letniej dziewczynki.

A case of rhabdomyosarcoma embryonal of the nasopharynx in a 3-year-old girl has been described. Clinical manifestations and initial diagnostic difficulties resulting in delay of proper diagnosis were discussed. Division of RMS, etiology, differentiation of the most often applied clinical sign and modern therapeutic opportunities depending on the location, histopathological type, the disease advancement level and the patient's age were described. Late outcome and the initially positive results of the employed treatment were emphasized in the presented case. The authors would like to underline the necessity to apply all imaging methods available (CT scan and MRI), in order to determine the exact site and extensiveness of malignancy which is crucial for evaluation of appropriate treatment and therapy.