{"title":"Alveolar soft part sarcoma in a child - a case report.","authors":"V Bartoš, D Sejnová, A Skálová, I Béder","doi":"10.48095/ccko2023396","DOIUrl":"10.48095/ccko2023396","url":null,"abstract":"<p><strong>Background: </strong>Alveolar soft part sarcoma (ASPS) is a very rare mesenchymal malignancy of uncertain origin. It mostly affects young people, with about a quarter of cases being diagnosed in children.</p><p><strong>Case: </strong>An 11-year-old girl had a painless subcutaneous \"lump\" in the left elbow area. Imaging exams revealed a solid soft-tissue intramuscular mass of suspicious appearance. A surgical excision of lesion was performed. The biopsy consisted of a lobular tumor measuring 35 × 20 × 12 mm. Histology revealed an epithelioid-cell population arranged in organoid pseudoalveolar pattern. It immunohistochemically expressed TFE3 and harbored the ASPSCR1:: TFE3 gene fusion. A diagnosis of ASPS was established. Subsequently, a wide re-excision of the scar was performed without microscopic residual tumor. The patient is currently without evidence of local recurrence or metastasis.</p><p><strong>Conclusion: </strong>ASPS is considered an aggressive and prognostically unfavorable chemoresistant neoplasm. Children have a better prognosis compared to adults. Early detection of tumor in a localized stage with complete surgical removal remains a mainstay therapeutic option. Due to its tendency to late metastases, a long-term thorough follow-up of the patient is necessary.</p>","PeriodicalId":35565,"journal":{"name":"Klinicka Onkologie","volume":"36 4","pages":"396-400"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50158941","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"How unknown is metastatic carcinoma to cervical lymph nodes from an unknown primary?","authors":"M Pála","doi":"10.48095/ccko2023364","DOIUrl":"10.48095/ccko2023364","url":null,"abstract":"<p><strong>Background: </strong>The term metastatic carcinoma to cervical lymph nodes from an unknown primary includes a small group of tumors that present themselves with metastases to the cervical nodes, and in which diagnostic methods do not reveal the primary source of these metastases. Histologically, in most cases, these are metastases of squamous cell carcinoma. Carcinomas of unknown primary metastatic to cervical nodes account for < 5% of carcinomas of unknown primary and < 5% of head and neck cancers. The optimal treatment has not yet been defined. In the absence of distant metastases, the intention of treatment is curative. Patients are treated mostly with combined approaches including surgery, radiotherapy, or concomitant chemoradiotherapy. Radiotherapy is part of the treatment algorithm in most of the referenced works and includes irradiation of the mucosal sites of the pharyngeal axis as a potential localization of the primary tumor and unilateral or, more often, bilateral irradiation of the neck. Due to the higher risk of late toxicities observed, individualization of irradiated volumes based on the extent of the disease or other clinical parameters is a rational way to reduce these risks. Purpose: The presented work discusses the treatment options for patients with metastatic carcinoma to cervical lymph nodes from an unknown primary. Furthermore, the work reports on the high effectiveness of curative radiotherapy in this group of tumors.</p>","PeriodicalId":35565,"journal":{"name":"Klinicka Onkologie","volume":"36 4","pages":"364-369"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"50158943","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The effect of demographic and clinical data on anxiety and depression levels in breast cancer patients receiving radiotherapy.","authors":"Y Benderli Cihan, O Öztürk","doi":"10.48095/ccko2023453","DOIUrl":"10.48095/ccko2023453","url":null,"abstract":"<p><strong>Purpose: </strong>The aim of this study is to investigate the effects of sociodemographic and clinical data on depression and anxiety levels in patients who undergoing radiotherapy for breast cancer.</p><p><strong>Materials and methods: </strong>A total of 111 patients with breast cancer treated in the Radiation Oncology Department of Kayseri City Education and Research Hospital were included in this study. The study was planned prospectively as a survey research based study. Ethics committee approval was obtained. After obtaining the necessary consent for voluntary participation, patients were interviewed face-to-face. The research survey included the Hospital Anxiety and Depression Scale (HADS), as well as demographic and clinical information. Statistical analysis was performed with the collected data.</p><p><strong>Results: </strong>According to the results of repeated measures analysis of variance, the mean difference between the 3-month and 6-month measurements of the depression scale values on the first day of radiotherapy was statistically significant (P < 0.001). Mean differences were statistically notable for age and marital status variables in anxiety and for age, education level, marital status, employment status, family history of cancer, menopause, surgery, chemotherapy and hormone therapy variables in depression. When the change in the presence (> 10 depression scale) or absence (< 10 depression scale) of depression at three different times of radiotherapy (first day, 3 and 6 months) was examined, a statistically notable difference was found between the depression scale values of patients receiving radiotherapy on the first day, 3 months after radiotherapy and 6 months after radiotherapy (P < 0.05).</p><p><strong>Conclusion: </strong>According to the results of our study, the psychological health of women with breast cancer was affected during and after radiotherapy. As a response, psychiatric counseling should be considered as a part of the treatment for depression and anxiety that occur during and after treatment in breast cancer patients.</p>","PeriodicalId":35565,"journal":{"name":"Klinicka Onkologie","volume":"37 6","pages":"453-461"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139075265","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
L Krasničanová, R Saade, P Priščáková, H Gbelcová, K Kaľavská, M Karaba, J Benca, M Mego, V Repiská
{"title":"Pilot study of gene mutations associated with Lynch syndrome in Slovak patients with breast cancer.","authors":"L Krasničanová, R Saade, P Priščáková, H Gbelcová, K Kaľavská, M Karaba, J Benca, M Mego, V Repiská","doi":"10.48095/ccko2023130","DOIUrl":"https://doi.org/10.48095/ccko2023130","url":null,"abstract":"<p><strong>Background: </strong>Lynch syndrome (LS) is an autosomal dominant inherited disorder which causes an increased risk of cancer, especially colorectal and endometrial carcinomas. Recent studies have shown an association between LS and breast cancer as well. The aim of our study is to highlight the possible presence of mutations in genes associated with LS in patients with breast cancer and the need to include the examination of Lynch-associated genes in patients with a family history of breast cancer as well as in patients with recurrent breast cancer, as well as with the occurrence of other Lynch-associated cancer.</p><p><strong>Materials and methods: </strong>We analyzed tumor tissue samples from 78 patients with primary breast cancer. Our samples were tested with a gene panel associated with the risk of developing breast cancer, while in our study we focused primarily on the occurrence of mutations in mismatch-repair genes. DNA isolated from tumor tissue was sequenced using next generation sequencing (NGS) and analyzed using the Ingenuity Variant Analysis tool. To confirm the germline mutation, we examined the patient's blood sample using NGS sequencing.</p><p><strong>Results: </strong>As a result of our analysis, we managed to identify a mutation in the PMS2 gene in one patient's breast tumor tissue. The presence of this mutation indicates that the resulting cancer may be a consequence of LS. As for pathogenicity, this was probably a pathogenic variant, as we detected deletions in the exon region, which led to frameshift mutation. Moreover, we also identified single-nucleotide pathogenic variants in the TP53 and PIK3CA genes. To definitively establish the diagnosis of LS in the patient, we examined a blood sample, where we also identified a mutation of the PMS2 gene.</p><p><strong>Conclusion: </strong>LS is underdiagnosed in many Lynch-associated cancers. However, in the case of a familial occurrence of breast cancer and other Lynch-associated genes, it is important to think about a possible diagnosis of LS and, if the patient meets the diagnostic criteria, to carry out a genetic examination of Lynch-associated genes.</p>","PeriodicalId":35565,"journal":{"name":"Klinicka Onkologie","volume":"36 2","pages":"130-134"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9788579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J Zavadil, T Rohan, J Juráček, I Kiss, L Ostřížková, V Válek, O Slabý, T Andrašina
{"title":"Biomarkers as prognostic and predictive factors in patients with hepatocellular carcinoma undergoing radiological oncological interventions.","authors":"J Zavadil, T Rohan, J Juráček, I Kiss, L Ostřížková, V Válek, O Slabý, T Andrašina","doi":"10.48095/ccko2023104","DOIUrl":"https://doi.org/10.48095/ccko2023104","url":null,"abstract":"<p><strong>Background: </strong>Hepatocellular carcinoma is the most common malignant liver tumor in adults and thermal ablation and transarterial embolization are important methods of therapy. Thermal ablation can be used in early stages. Methods based on the transarterial approach, especially transarterial chemoembolization, play an important role in intermediate stage diseases. The success of procedures depends not only on the biological nature and the size of the tumor, on the technical design of the procedure and on the patient's response to treatment, but also on the molecular changes associated with these procedures. In addition to classic predictive and prognostic factors including age, patient comorbidities, Child-Pugh score, tumor characteristics, presence of large surrounding vessels, and portal vein thrombosis, molecular prognostic and predictive factors (serum biomarkers) are often mentioned in studies. Currently, only a-fetoprotein is routinely used as a prognostic biomarker; however, there are studies referring to new serum biomarkers that can potentially help to classical markers and imaging methods to determine the cancer prognosis and predict the success of therapy. These biomarkers most often include g-glutamyltranspeptidase, des- g-carboxyprothrombin, some types of microRNAs, inflammatory and hypoxic substances, whose serum levels are changed by the intervention therapies. Evaluation of these molecules could lead to the optimization of the medical intervention (choice of therapy method, timing of treatment) or change the management of patient follow-up after interventions. Although several biomarkers have shown promising results, most serum biomarkers still require validation in phase III studies.</p><p><strong>Purpose: </strong>The aim of this work is to present a comprehensive overview of classical and molecular biomarkers that could potentially help in the prognostic stratification of patients and better predict the success and effect of radiological intervention methods.</p>","PeriodicalId":35565,"journal":{"name":"Klinicka Onkologie","volume":"36 2","pages":"104-111"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9788574","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Liver adenomatosis mimics metastatic liver involvement on FDG-PET/ CT.","authors":"O Bělohlávek, M Jarůšková, M Šmakal","doi":"10.48095/ccko2023143","DOIUrl":"https://doi.org/10.48095/ccko2023143","url":null,"abstract":"<p><strong>Background: </strong>Liver adenomatosis is a very rare disease. In the literature, we were able to find only two case reports documenting the appearance of this disease on PET/CT with 18F-fludeoxyglucose (FDG-PET/CT).</p><p><strong>Case: </strong>Numerous liver foci were detected during sonography in a 52-year-old female patient with uncharacteristic pain in the epigastrium without oncological history, with negative oncomarkers and without clinical signs of generalized neoplasia. Complementary MRI examination expressed the suspicion of metastatic origin of the foci, and FDG-PET/CT was indicated in order to identify the primary tumour and assess the extent of the disease. A whole-body FDG-PET/CT examination showed multiple (> 20) markedly hypermetabolic liver foci with 3-20 mm in diameter, reaching a relative accumulation of SUVBWmax = 13, together with several ametabolic cysts; elsewhere in the scope of the examination, focally pathologically increased metabolic activity was not evident. Subsequently, the patient underwent a biopsy targeted at one of the hypermetabolic liver foci with the finding of HNF 1A inactivated variant of hepatocellular adenoma; primary or secondary malignancy was not demonstrated. Considering the histological findings and the large number of liver foci, the final diagnosis of liver adenomatosis was set. The patient remains under continuous observation.</p><p><strong>Conclusion: </strong>Adenomatous foci were markedly hypermetabolic during FDG-PET/CT examination and could not be distinguished from tumour metastases by this method. Our finding is consistent with two other observations we were able to find in the literature.</p>","PeriodicalId":35565,"journal":{"name":"Klinicka Onkologie","volume":"36 2","pages":"143-145"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9788576","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Unique natural history of an EGFR mutated adenocarcinoma.","authors":"S Okauchi, H Satoh","doi":"10.48095/ccko202371","DOIUrl":"https://doi.org/10.48095/ccko202371","url":null,"abstract":"<p><strong>Background: </strong>Supportive care alone cannot be indicated for cancers for which established standard therapy exists unless there is a specific reason. Due to the refusal of standard therapy by the patient after proper explanation of the therapy, we experienced a long-term follow-up of >10 years with supportive care alone in an epidermal growth factor receptor (EGFR) mutated lung cancer patient.</p><p><strong>Case: </strong>A 70-year-old woman was referred due to the right lung with some ground glass opacities (GGOs). One of the GGOs which was resected in another hospital had been confirmed to be EGFR mutation-positive lung adenocarcinoma. Although EGFR-tyrosine kinase inhibitor (TKI) was explained to be the standard therapy, the patient refused receiving the therapy and wished to follow up imaging of the remaining GGOs. During the follow-up period of 13 years, the each GGO showed a gradual increase. The doubling time of the largest GGO and that of serum carcinoembryonic antigen was > 2,000 days, respectively.</p><p><strong>Conclusion: </strong>Although very rare, some of EGFR mutated lung adenocarcinoma might have a very slow progression. Clinical course of this patient provides useful information to the clinical practice of future patients who may have similar clinical courses.</p>","PeriodicalId":35565,"journal":{"name":"Klinicka Onkologie","volume":"36 1","pages":"71-74"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9393224","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Predictive biomarkers of response to immunotherapy in triple-negative breast cancer - state of the art and future perspectives.","authors":"V Tancoš, A Blichárová","doi":"10.48095/ccko202328","DOIUrl":"https://doi.org/10.48095/ccko202328","url":null,"abstract":"<p><strong>Background: </strong>Immunotherapy by using immune checkpoint inhibitors (ICIs) heralded a new era in the treatment of patients with advanced triple-negative breast cancer (TNBC). Nevertheless, in a substantial proportion of TNBC patients, the clinical outcomes of ICIs treatment remain unpredict-able and proper bio-markers to identify tumors sensitive to immunotherapy are urgently needed. Currently, the most clinically relevant bio-markers used to predict efficacy of ICIs in patients with advanced TNBC remain the immunohistochemical analysis of programmed death-ligand 1 (PD-L1) expression, the assessment of tumor infiltrating lymphocytes (TILs) present in the tumor microenvironment (TME), and the evaluation of the tumor mutational burden (TMB). Emerging bio-markers related to activation of the transforming growth factor beta signaling pathway, the discoidin domain receptor 1, and thrombospondin-1 as well as other cellular and molecular factors present within TME, have the potential to be utilized as predictors of response to ICIs in the future.</p><p><strong>Purpose: </strong>In this review, we summarize the current knowledge of mechanisms regulating PD-L1 expression, of the predictive value of TILs as well as of associated cellular and molecular components present in the TME in TNBC. Furthermore, TMB and emerging bio-markers with potential value in predicting efficacy of ICIs are discussed, and new therapeutic strategies will be outlined.</p>","PeriodicalId":35565,"journal":{"name":"Klinicka Onkologie","volume":"36 1","pages":"28-34"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9409727","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Ethnic differences in the incidence of childhood cancer.","authors":"P Bician, M Mego","doi":"10.48095/ccko2023192","DOIUrl":"https://doi.org/10.48095/ccko2023192","url":null,"abstract":"<p><strong>Background: </strong>In 2020, 19,292,789 new malignancies were diagnosed worldwide (including non-melanoma skin cancer), causing 9,958,133 deaths. Europe, which accounts for 9% of the world's population, accounts for up to 22.8%, with 4.4 million new cases and 1.96 million deaths. Children and adolescents aged 0-19 account for only about 1% of the overall cancer incidence. Differences between adults and children with cancer include not only incidence but also mortality (approx. 45% at the age of ≥ 20 years compared to 15% at the age < 19 years), and individual types of cancer (breast, colon and lung tumors at the age > 20 years, and leukemia, central nervous system tumors and lymphomas at the age of.</p>","PeriodicalId":35565,"journal":{"name":"Klinicka Onkologie","volume":"36 3","pages":"192-197"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9676583","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Z Adam, L Pour, D Zeman, M Krejčí, I Boichuk, M Krejčí, M Štork, V Sandecká, Z Král
{"title":"Waldenström's macroglobulinemia - clinical symptoms and review of therapy yesterday, today and tomorrow.","authors":"Z Adam, L Pour, D Zeman, M Krejčí, I Boichuk, M Krejčí, M Štork, V Sandecká, Z Král","doi":"10.48095/ccko2023177","DOIUrl":"https://doi.org/10.48095/ccko2023177","url":null,"abstract":"<p><strong>Background: </strong>Waldenström macroglobulinemia (WM) is a lymphoplasmocytic lymphoma with immunoglobulin M monoclonal protein. The incidence of this disease is very low (0.4/100,000), so that this disease can be regarded as an orphan's disease. It means that new drugs are often tested and registered for more frequent diseases.</p><p><strong>Purpose: </strong>In this review we will focus on the efficacy of the new drugs for WM.</p><p><strong>Results: </strong>The current treatment options for symptomatic WM patients include alkylating agent cyclophosphamide and anti-CD20 monoclonal antibodies. Therapy with rituximab and bendamustin resulted in longer therapeutic response then therapy with rituximab, cyclophosphamide and dexamethasone. Many drugs, used in multiple myeloma (MM), shoved promising results in WM patients. Bortezomib is effective in WM, but its neurotoxicity is higher in WM than in MM patients. Therefore, new proteasome inhibitors, carfilzomib and ixazomib, are better tolerated as documented in several studies. New types of antiCD20 antibody (obinutuzumab) can be used in patients with rituximab intolerance. in five of our patients with WM, obinutuzumab and bendamustin reached deeper responses than therapies administered in previous lines of therapy. Oral Bruton tyrosine kinase (BTK) inhibitor ibrutinib alone and in combination with rituximab have extended the treatment options for WM patients. New BTK inhibitors (e. g. acalabrutinib, zanubrutinib, and vecabrutinib) were tested and their lower toxicity (atrial fibrillation) was documented. Moreover, the BCL2 inhibitor venetoclax is newly tested.</p><p><strong>Conclusion: </strong>New antiCD20 antibody (obinutuzumab) is of advantage in patients with WM with rituximab intolerance as well as bendamustin and new proteasome inhibitors (ixazomib and carfilzomib) or new BTK inhibitors with lower cardiotoxicity. Many of the abovementioned drugs do not have official registration for WM and can be administrated with the consent of the health care provider only. Thus, this work brings evidence of their efficacy.</p>","PeriodicalId":35565,"journal":{"name":"Klinicka Onkologie","volume":"36 3","pages":"177-191"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9676584","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}