Antoni Krzeski

Rhabdomyosarcoma of the head and neck in children.

Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma in children. It is localized in the head and neck region in 40% of cases. Treatment of RMS is complex, including multi-drug chemotherapy, radiotherapy and surgery. The progress that has been accomplished in oncology in recent decades significantly improved outcomes. The 5-year survival rate raised from 25% in 1970 to 73% in 2001, according to IRS-IV data. The outcome is influenced by primary tumor localization, clinical staging, histological tumor type and age at the moment of diagnosis. The relatively rare incidence of these tumors resulted in difficulties in creating more standardized therapeutic protocols. Comparison of outcomes in large patients groups led to an increase in the number of patients with complete remission. Although survival rates of RMS patients have improved, searching for new therapeutic modalities and substances is still essential to improve outcomes in cases of more advanced stages and unfavorable tumor localizations.

PRAME expression in head and neck cancer correlates with markers of poor prognosis and might help in selecting candidates for retinoid chemoprevention in pre-malignant lesions

OBJECTIVES PRAME (Preferentially Expressed Antigen in Melanoma) is a tumor-associated antigen recognized by immunocytes, and it induces cytotoxic T cell-mediated responses in melanoma. PRAME expression in tumors interferes with retinoic acid receptor (RAR) signaling thus promoting tumor progression. Here, we study PRAME expression in head and neck squamous cell carcinoma (HNSCC) to determine its potential clinical significance. MATERIALS AND METHODS PRAME expression in HNSCC was evaluated by immunohistochemistry in tissue microarrays of primary tumors (n=53), metastatic lymph nodes (n=8) and normal oral mucosa (n=11). Biopsies of dysplastic oral lesions (n=12) were also examined. PRAME expression levels in tissues were correlated with markers of poor prognosis in HNSCC. PRAME mRNA in HNSCC cell lines and in normal immortalized human keratinocytes (HaCaT cell line) was measured by qRT-PCR, and the protein expression by flow cytometry and western blots. RESULTS PRAME was expressed in HNSCC cell lines and HNSCC lesions. PRAME expression in dysplastic mucosa was variable. No or only weak expression was found in normal cells or tissues. PRAME expression levels significantly correlated with the tumor grade, size, nodal involvement and the clinical status of HNSCC patients. CONCLUSIONS Elevated PRAME expression associates with clinicopathologic markers of poor outcome in HNSCC and might identify potential candidates with pre-cancerous lesions for chemoprevention with retinoids.

Extent of pathological changes in the paranasal sinuses of patients with cystic fibrosis: CT analysis.

The aim of the study was to define the characteristic changes revealed by computed tomography (CT) examination of the paranasal sinuses in patients with cystic fibrosis (CF). The group of 30 CF patients was evaluated in the outpatient clinic of the Department of Otorhinolaryngology, Medical University of Warsaw, from 1996 to 1998. The control group consisted of 30 patients with chronic rhinosinusitis (CRS). CT scans were obtained from both groups of patients, and findings revealed more advanced pathological changes in the CF than CRS group. The extensive inflammatory process observed in CT scans of CF patients resulted in the impairment of frontal and maxillary sinus development, destruction of bony structures, and medial projection of the lateral nasal wall.

Anatomic variations of the lateral nasal wall in the computed tomography scans of patients with chronic rhinosinusitis

The development of computed tomography (CT) technique results in the advance in visualization of the anatomic structures of the lateral nasal wall. It allows the anatomic variations of this region to be identified precisely, which is important in surgical treatment of chronic rhinosinusitis. The aim of this study was to determine the incidence of anatomic variations of the lateral nasal wall in a group of patients with chronic rhinosinusitis. The preoperative, frontal plane CT scans of the study group were evaluated using the method of the four-zone interpretation. The incidence of the anatomic variations of the lateral nasal wall was consistent with the results reported by other authors.

Cystic fibrosis in rhinologic practice.

Background Cystic fibrosis (CF) is the most common genetic lethal disorder that affects white populations. Chronic rhinosinusitis (CRS) with extensive nasal polyposis is one of the manifestations of CF. Methods The aim of this study was to determine the prevalence and extent of CRS in CF patients. Results The study indicated that the signs and symptoms of CRS were present in all patients with CF and they were more advanced than in the control group. The most severe inflammatory changes in the paranasal sinuses were detected in patients with the dF508 gene mutation type who suffered from CRS for >3 years. Conclusions Massive nasal polyposis, dilated base of the nose, mucociliary clearance impairment, and significant radiological changes (frontal and maxillary sinus hypoplasia, bony destruction, and medial bulging of the lateral nasal wall) were identified to be the characteristic signs of the CRS in CF patients.

The correlation of TAS2R38 gene variants with higher risk for chronic rhinosinusitis in Polish patients.

BACKGROUND Bitter taste receptors (T2Rs), especially T2R38s appear as innovative regulators of innate immunity in the respiratory system. The single nucleotide polymorphisms (SNPs) in TAS2R38 gene may contribute to individual differences in susceptibility to respiratory infections especially chronic rhinosinusitis (CRS). TAS2R38 genotypes distribution varies by geographic region, race and ethnicity. The aim of the preliminary study was the identification of SNPs in TAS2R38 encoding genes in Polish patients with CRS and finding potential correlation with CRS phenotypes. MATERIAL AND METHODS The preliminary study contained 20 CRS patients undergoing functional endoscopic sinus surgery (FESS). Fresh sinus mucosa (SM) was obtained during FESS in CRS patients. Patients were genotyped for TAS2R38 using Sanger method and the genotype occurrences of the clinically recalcitrant CRS cohort was evaluated. Analysis of TAS2R38 expression in SM of CRS patients was performed using immunohistochemistry (IHC). RESULTS T2R38 was highly expressed in SM of CRS patients. Patients with CRS demonstrated both common genotypes PAV, AVI. The heterozygotes frequency (AVI/PAV) was the highest. The protective genotype (PAV/PAV) was noticed in the lowest frequency and connected with lower average value of CT score compare to AVI/AVI genotypes (p=0.01). CONCLUSIONS The work presented in this study provides the hypothesis that airway bitter T2Rs are an innovative sphere of human respiratory innate protection. TAS2R38 polymorphism may influence the susceptibility to CRS. The AVI haplotypes are an independent risk factors for CRS. Additionally, the bitter taste receptors and related signalling pathways might create an unique group of therapeutic targets to treat CRS.

Endoscopic transnasal management of inverted papilloma involving frontal sinuses

Inverted papilloma is a benign locally aggressive tumor of paranasal sinuses which has been traditionally managed with external surgical approaches. Advances in tumor imaging, surgical instrumentation and intraoperative visualization have led to a gradual shift to endonasal attachment-oriented surgery. Involvement of both frontal sinuses by inverted papilloma is rare. There are scant reports in the literature regarding this topic. We present 2 cases of the tumor involving both frontal sinuses removed by median drainage (Draf III procedure) under endoscopic guidance without any additional external approach. The whole cavity of both frontal sinuses was easily inspected at the end of the procedure. No early or late complications were observed. No recurrence was seen in 1-year or 2-year follow-up. Management of frontal sinus inverted papilloma with the endoscopic median drainage approach is feasible and seems to be effective.

Operacje z dostępu Draf III w materiale Kliniki Otolaryngologii WUM

Summary During last decades Draf III procedure gained popularity in treatment of different pathologies of the frontal sinus such as chronic sinusitis or benign tumors. We present a series of 10 patients treated with this procedure from a 2 year-period. Indications included chronic rhinosinusitis – four patients, frontal sinus mucocele – four patients (one with destruction of the posterior table), osteoma – one patient (stage 3 according to Kennedys grading system), and inverted papilloma – one patient (Krouse T3 lesion). Observation period ranged from 4 months to 2 years. Results There were no early complications. Gradual narrowing of the created ostium was observed in all of the patients. This led to total obstruction in two, and stenosis (not allowing for passing with 4 mm endoscope) in another two patients. The patient after inverted papilloma removal showed no recurrence in 11 months follow-up. Conclusion Draf III procedure is alternative for external approach that can be used for treatment of chronic rhinosinusitis and benign frontal sinus tumors of different size.

Spontaneous sphenoid sinus cerebrospinal fluid leak and meningoencephalocele – are they due to patent Sternberg's canal?

Sternbergs canal is a congenital bony defect in the lateral wall of the sphenoid sinus. If it persists to adulthood, it may become a source of spontaneous cerebrospinal fluid leak (CSF) and meningoencephalocele. The aim of the study was to describe the authors’ experience and review articles related to spontaneous sphenoid sinus CSF leaks and Sternbergs canal. We analysed patients managed surgicallly due to sphenoid sinus CSF leak and performed a PubMed database search. Two female patients with spontaneous CSF leak of sphenoid origin were found. Both patients underwent surgery with the endoscopic endonasal approach, and the defect was closed using the multi-layer technique. Twelve articles related to CSF leaks of sphenoid origin (due to Sternbergs canal) were found in the PubMed database. Lines of lesser resistance within sphenoid bone may underlie CSF leak pathology together with intracranial hypertension. The endoscopic transnasal approach to the sphenoid sinus is an excellent alternative to standard transcranial procedures.

Tracheal adenoid cystic carcinoma mimicking a thyroid tumor: A case report

At present, only eight cases of tracheal adenoid cystic carcinomas (ACCs) mimicking thyroid tumors have been reported. Since there are no guidelines available regarding their diagnosis and treatment, they present a significant clinical challenge. In the present study, patient treatment was analyzed to deliver the first concise summary of treatment options in patients with ACC mimicking a thyroid tumor. In addition, all available data regarding molecular abnormalities of this disease have been discussed. The current study presents a case of a 17-year-old patient with a tracheal ACC mimicking a thyroid tumor. The patient was diagnosed in 2007 with a pathological mass between the left lobe of the thyroid and the trachea, and underwent surgery and radiotherapy. In 2010, multiple lesions in the lungs were diagnosed and pulmonary metastasectomy was performed. Following surgery, the patient has been disease-free for almost 30 months. Thyroid tumor biopsy may reveal ACCs. This pathological report requires further investigation of the head and neck in order to confirm if the disease is of tracheal origin. Patients may present with a neck swelling, hoarseness of voice or dysphagia. Surgery must be considered as first-line therapy for all patients with local disease as it may be curative. For palliative treatment chemoradiotherapy based on cisplatin may be effective. The identification of cytogenetics, tumor suppressor genes, oncogenes, epigenetic alterations and mitochondrial abnormalities specific for ACCs is critical to the development of targeted therapies. Thus far, large studies have only reported the transcriptional activator Myb and mammalian target of rapamycin signaling pathway to be disrupted in ACCs.