Transient abnormal myelopoesis and acute leukemia in children with Down syndromeMain topic

Danica Zapletalová, Martin Baláž, Hana Bernatíková, Terézia Tureková

Onkologie. 2023:17(5):308-311 | DOI: 10.36290/xon.2023.057  

Down syndrome is one of the best known and most common chromosomal aberrations. The presence of excess genetic material of chromosome 21 causes multiorgan impairment. From an oncological point of view, the predisposition of these individuals to hematological malignancies in childhood is especially important. Trisomy of chromosome 21 itself leads to perturbed hematopoiesis and it is the first step in the development of transient abnormal myelopoesis, a unique clonal disease that occurs only in the presence of trisomy of chromosome 21. It is a preleukemic phase with variable clinical manifestations from a completely asymptomatic course to a life-threatening...

Extremely rare spinal tumor in Li-Fraumeni syndromeMain topic

Pavel Tinka, Zdeněk Pavelka, Klára Vejmělková, Marta Ježová

Onkologie. 2023:17(5):304-307 | DOI: 10.36290/xon.2023.056  

The aim of this article is to describe a case of the infrequent spinal adenoma in paediatric patient. This adenoma was resected with inoperable residuum and histology confirmed an extremely rare spinal adrenocortical adenoma. Tumor was was presented by symptoms of spinal compression and endocrinological signs of pseudopubertas praecox. There was not any other lesion found via PET/MRI examination. Tumor was not accidental, patient inherited pathological allele of the gene coding TP53 protein from the father line, where was frequent occurrence of malignancies in early age. Understanding principles of Li-Fraumeni syndrome is crucial to assess the effective...

Nové trendy v léčbě pokročilého kolorektálního karcinomuEditorial

MUDr. Zdeněk Linke

Onkologie. 2023:17(4):219  

Effect of immunotherapy and radiotherapy in the treatment of Merkel cell carcinoma in a patient with myelodysplastic syndromeCase report

Miroslav Důra, Ivana Krajsová, Jolana Mertová

Onkologie. 2023:17(4):290-294 | DOI: 10.36290/xon.2023.055  

The authors present a case of a complete treatment response in a patient treated for metastatic Merkel cell carcinoma with a combination of radiotherapy and immunotherapy with avelumab. Immunotherapy was terminated prematurely due to relapse of myelodysplastic syndrome. Despite premature termination of immunotherapy, complete disease remission has been sustained. Clinical and histopathological properties of Merkel cell carcinoma and the recommended treatment algorithm are discussed. Attention is paid to the management of avelumab treatment.

Nivolumab in the treatment of pretreated patients with metastatic colorectal carcinoma with high microsatellite instabilityCase report

Beatric Bencsiková

Onkologie. 2023:17(4):287-289 | DOI: 10.36290/xon.2023.054  

Nivolumab in combination low-dose ipilimumab has provided a robust and long-term clinical benefit with a manageable safety profile in previously treated patients with metastatic colorectal carcinoma (mCRC) harboring high microsatellite instability/mismatch repair protein deficiency (MSI-H/dMMR) with a median follow-up of 13.4 and 25.4 months in the phase-2 multicohort CheckMate 142 study. The 5-year follow-up data on the efficacy and safety were presented at ASCO 2022. The effect of treatment has also been confirmed by clinical experience and a particular patient case.

The use of immunotherapy and targeted therapy in Richter's transformationCase report

Tereza Líkařová, Martin Špaček

Onkologie. 2023:17(4):282-286 | DOI: 10.36290/xon.2023.053  

Richter´s syndrome (RS) is a serious complication of chronic lymphocytic leukemia, occurring yearly in 2-10 % of cases. Despite new treatment modalities in the field of haematooncology, RS remains a major clinical challenge. This case report presents a patient in whom disease control was not achieved despite the use of various novel therapeutic approaches. This case thus demonstrates the extremely unfavorable prognosis of RS patients and the clinical urgency of developing new therapeutic approaches.

Modern approach to the treatment of chronic lymphocytic leukemiaReview articles

Lukáš Smolej

Onkologie. 2023:17(4):273-281 | DOI: 10.36290/xon.2023.052  

Chronic lymphocytic leukemia (CLL) is the most common lymphoid malignancy of adults in the Euro-American population and predominantly affects the elderly: the median age at diagnosis is between 65 and 72 years. Most patients are nowadays diagnosed at an early asymptomatic stage and do not require treatment. The heterogeneity of the prognosis of CLL is extraordinary. Even with long-term follow-up, about 50% of CLL patients do not progress and never require treatment, which is initiated only in case of the disease´s clinical activity. Prognostic factors, especially TP53 gene deletion/mutation, other cytogenetic abnormalities, and mutation status...

Brentuximab vedotin consolidation therapy after autologous stem cell transplantation in relapsed/refractory classic Hodgkin lymphoma patients in the Czech RepublicReview articles

Jozef Michalka, Zdeněk Král, Jana Marková, Ľubica Gahérová, Mária Maco, Vít Procházka, Andrea Janíková, Jan Kořen, Alice Sýkorová, Pavla Štěpánková, Kateřina Steinerová, Lekaa Mohammad, Juraj Ďuraš, Barbora Velacková, Tomáš Doležal, Kateřina Benešová, Marie Lukášová, Heidi Móciková

Onkologie. 2023:17(4):267-272 | DOI: 10.36290/xon.2023.051  

The results from the clinical trial AETHERA showed that consolidation therapy with brentuximab vedotin after high-dose chemotherapy followed by autologous stem cell transplantation increases progression free survival in high-risk patients with relapsed/refractory classic Hodgkin lymphoma. In this multicenter retrospective analysis from seven hematological centers in the Czech republic we have analysed real-life data of 39 patients treated between January 2015 and December 2021. Brentuximab vedotin was not administered in the previous treatment and the patients were at increased risk of subsequent lymphoma relapse with at least one defined risk factor....

Cemiplimab: monotherapy and combination therapy of NSCLC - analysis of locally advanced diseaseReview articles

Marie Drösslerová

Onkologie. 2023:17(4):261-266 | DOI: 10.36290/xon.2023.050  

Patients with unresectable locally advanced NSCLC who are not candidates for concurrent chemoradiation have been excluded from trials with immunotherapy. Trials with cemiplimab are exception. Trials EMPOWER-Lung 1 (first-line cemiplimab monotherapy) and EMPOWER-Lung 3 (first-line cemiplimab with chemotherapy) were not only focused on metastatic stage but also included patients mentioned above.

Current status of nephrectomy in the treatment of metastatic renal cell carcinomaReview articles

Vladimír Šámal, Igor Richter, Vít Paldus, Marie Pechová

Onkologie. 2023:17(4):257-260 | DOI: 10.36290/xon.2023.049  

Renal cell carcinoma (RCC) accounts for approximately 3% of all malignant tumors, and 15% of tumors are metastatic at time of diagnosis. For many years,  cytoreductive nephrectomy was the mainstay of treatment. For the patients with metastatic disease, cytoreductive nephrectomy (CN) is predominantly a palliative procedure and systemic therapy is essential. Adrenalectomy as a part of CN is not considered as a standard part of the procedure except for obvious tumor invasion into the adrenal gland. Lymfadenectomy as a part of CN has only staging effect. Results of cytokine study comparing CN plus interferon-based immunotherapy vs. interferon immunotherapy...

Role of ruxolitinib in treatment of polycythemia veraReview articles

Natália Podstavková

Onkologie. 2023:17(4):252-255 | DOI: 10.36290/xon.2023.048  

Ruxolitinib, selective oral inhibitor of Janus kinases JAK1/JAK2, is approved for therapy of primary myelofibrosis but also as a second line therapy of patients with polycythemia vera which are resistant or intolerant to hydroxyurea. Randomized studies RESPONSE and RESPONSE-2 have shown its superiority in achieving hematological remission and decreased symptom burden when compared to best available therapy. MAJIC-PV study demonstrated in PV benefits of ruxolitinib in superior event-free survival and prolonged overall survival of patients.

Neo-/adjuvant immunotherapy and targeted therapy of non-small cell lung cancerReview articles

Martin Svatoň, Jiří Blažek

Onkologie. 2023:17(4):246-251 | DOI: 10.36290/xon.2023.047  

For many years, operable non-small cell lung cancer has been associated with a high percentage of recurrences with a limited possibility of neo-/adjuvant treatment in the form of chemotherapy alone. However, recent years have seen great development in this field. The first phase III studies yielded positive data for the adjuvant use of osimertinib, atezolizumab and pembrolizumab. Neoadjuvant use of nivolumab together with chemotherapy and perioperative approach with pembrolizumab and chemotherapy also brought other positive results. The aim of this article is therefore to bring a summary of the changes and new findings in this area and to point out...

Treatment of metastatic colorectal carcinoma - 3^rd line and aboveMain topic

Pavel Pacas, Adam Lukáč

Onkologie. 2023:17(4):241-245 | DOI: 10.36290/xon.2023.046  

Terapeutic possbilities in mCRC in 3rd and higher line of treatment are constantly expanding not only by new combinations of actual treatment possibilities, but also by discoveries of new small targeted molecules. Results of clinical studies prove that by right therapeutic intervention, we can prolong lives of highly pre-treated patients without worsening their quality of life. Ever evolving clinical oncology require us to have constant knowledge of actual therapeutic regimes and formation of new approach in higher lines of treatment in advanced illness.

Options and rationale for reinstituting anti-EGFR therapy in pretreated RAS wild-type advanced colorectal carcinomasMain topic

Zdeněk Linke

Onkologie. 2023:17(4):233-240 | DOI: 10.36290/xon.2023.045  

Failure of anti-EGFR therapy in advanced RAS wild-type colorectal carcinoma at the initial lines of treatment may not necessarily mean its permanent and definitive inefficacy. One option is retreatment with anti-EGFR based therapies after previous progression (i. e., rechallenge). Another option is to reinitiate anti-EGFR treatment after previous discontinuation for a reason other than progression (i. e., reintroduction). Alternatively, sequential treatment can be used, i. e., switching between various anti-EGFR agents. A last option is to reinstitute anti-EGFR treatment in an effort to overcome the resistance of anti-EGFR therapy by dose escalation...

Treatment of BRAF V600E-mutated metastatic colorectal cancerMain topic

Jiří Tomášek

Onkologie. 2023:17(4):230-232 | DOI: 10.36290/xon.2023.044  

Colorectal cancers can be divided into several subgroups according to their molecular characteristics. A small subgroup comprises cancers with a BRAF mutation. The negative prognostic significance of this mutation in metastatic colorectal cancer (mCRC) has long been known; in recent years, we have made use of the fact that it is also a strong predictor of the efficacy of combined targeted therapy with cetuximab + encorafenib. The present paper deals with the treatment of patients with mCRC and BRAF V600E mutation.

Recent advances in immunotherapy for metastatic colorectal cancerMain topic

Stanislav Batko

Onkologie. 2023:17(4):224-229 | DOI: 10.36290/xon.2023.043  

Immunotherapy has a established role in the treatment of metastatic colorectal cancer in patients with microsatellite instability/deficiency in mismatch repair genes accounting for roughly 5% of all metastatic colorectal cancers. Data from smaller studies also strongly support similarly good efficacy in tumors with the POLE1/POLD1 mutation. Intensive research is underway to break down the resistance to immunotherapy observed in microsatellite stable colorectal cancers. Some smaller non-randomized studies suggest a possible benefit of combining immunotherapy with multikinase inhibitors or chemotherapy and targeted treatment in the population of microsatellite...

Všechno, co jste kdy chtěli vědět o karcinomu prsu u mladých žen, ale báli jste se zeptatEditorial

MUDr. Markéta Palácová

Onkologie. 2023:17(3):135  

Léčba pacientů s mnohočetným myelomem po expozici lenalidomidu a postavení přípravku Ninlaro v terapeutické sekvenciHeard at a congress

Dr. Karthik Ramasamy

Onkologie. 2023:17(3):209-214  

Patient with upgrading gangliogliomaCase report

Marek Šlachta, Ondřej Kalita, Dominik Hraboš, Lumír Hrabálek, Martin Doležel, Jiří Drábek, Miroslav Vaverka

Onkologie. 2023:17(3):205-208 | DOI: 10.36290/xon.2023.041  

Ganglioglioma is a rare primary brain tumor. It is commonly located supratentorialy, in the temporal lobe (>70%). The main clinical manifestation is a drug-resistant epileptic seizures. A rare, anaplastic, form is considered based on histological anaplastic signs of the glial component. Our patient with WHO grade 1 ganglioglioma underwent several surgeries for the gradual neoplasm progression. The histological examination shows the tumor development and its gradual up-grading into an anaplastic form (WHO grade 3), of which biology corresponded with the IDH wild-type glioblastoma. Current molecular-genetic tumor profiling is underpinnings for predilection...

Treatment with trifluridine/tipiracil in the third line of treatment for metastatic colorectal carcinoma in a patient with symptoms of toxicity during previous systemic treatmentCase report

Eva Čmuchařová

Onkologie. 2023:17(3):201-204 | DOI: 10.36290/xon.2023.040  

The following case report confirms the efficacy and good tolerability of trifluridine/tipiracil in a pretreated patient with mCRC who poorly tolerated previous systemic treatment.

Minimal invasive treatment and active surveillance of renal cell carcinomaReview articles

Marie Pechová, Vladimír Šámal, Vít Paldus, Igor Richter, Miroslav Šercl

Onkologie. 2023:17(3):197-200 | DOI: 10.36290/xon.2023.039  

Small Renal Masses (SRM) are defined as incidentally diagnosed, circumscribed solid or cystic tumors of the kidney < 4 cm with signs of saturation on a contrast-enhanced CT scan, corresponding to stage cT1aN0M0. In up to 60% of patients, the kidney tumor is detected incidentally. The incidence of SRM in elderly and polymorbid patients is also increasing. The standard of care for a localized kidney tumor is surgical treatment. In elderly patients or patients with serious comorbidities, SRM can only be actively monitored (Active Surveillance - AS). It is known that surgical treatment in the population over 75 years of age does not lead to a prolongation...

Bruton's tyrosine kinase inhibitors and their current status in the treatment of chronic lymphocytic leukemiaReview articles

Tereza Líkařová, Martin Špaček

Onkologie. 2023:17(3):192-196 | DOI: 10.36290/xon.2023.038  

Bruton's tyrosine kinase inhibitors (BTKi) are small molecules that interfere with the B-cell receptor signaling pathway and have an anti-tumor effect in chronic lymphocytic leukemia. Since the approval of ibrutinib in 2014, together with other targeted drugs, they have almost completely replaced standard chemotherapy regimens in relapsed disease and in the group of patients with proven TP53 aberrations are also preferred in the first-line setting. In addition to ibrutinib, a second-generation BTKi with a lower risk of cardiac adverse events, acalabrutinib, is now available in the Czech Republic. A number of other third-generation agents are in clinical...

Rechallenge with anti-EGFR therapyReview articles

Marián Liberko, Renata Soumarová

Onkologie. 2023:17(3):188-191 | DOI: 10.36290/xon.2023.037  

Continuum of care is a concept of treatment in which periods of aggressive, induction chemotherapy are followed by maintenance treatment with the aim to prolong overall survival of mCRC patients. Rechallenge with anti-EGFR drugs is a part of this concept. With this strategy, we may achieve significant response and control of disease, even in later lines of treatment, in selected group of patients. The aim of this review is to provide an overview of rechallenge with anti-EGFR therapy in the treatment of mCRC.

Tolerability of antidepressants and mechanisms of their adverse effects with regard to their use in cancer patientsReview articles

Jan Juřica

Onkologie. 2023:17(3):180-187  

Antidepressants represent an essential and effective therapeutic approach in treating depressive disorders and many other neuropsychiatric diseases. This review article summarises the most common adverse effects of the most commonly used antidepressants in the Czech Republic with respect to the frailty of cancer patients and their specific needs. These side effects usually have a completely logical explanation based on their receptor profile or pharmacokinetics. In this context, the advantages and disadvantages of these selected most commonly used antidepressants in cancer patients are summarised, especially regarding their tolerability and their desirable...

Small cell lung cancer, current treatment and perspectivesReview articles

Miloš Pešek

Onkologie. 2023:17(3):175-179 | DOI: 10.36290/xon.2023.035  

Small cell lung cancers are among cancer diseases in which treatment outcomes have improved very little during the previous decades. In recent years, however, there has been some improvement in early as well as long-term treatment outcomes in patients with extensive-stage disease. Clinical trials using the combination of standard chemotherapy of etoposide and a platinum derivative with durvalumab, atezolizumab, and pembrolizumab have yielded positive results. The results of trials mentioned here which focused on epigenetic changes in tumour tissue and on investigating gene expression profiles hold promise in terms of possible personalization of systemic...

Breast cancer and pregnancyMain topic

Marta Krásenská

Onkologie. 2023:17(3):169-174 | DOI: 10.36290/xon.2023.034  

The coincidence of breast cancer and pregnancy is increasing due to the postponement of childbearing until later in life. This article describes the newly proposed division into breast cancer in pregnancy and breast cancer in the postpartum period, which differ in biological characteristics and prognosis. Pregnancy after treatment for breast cancer has long been considered inappropriate, especially in patients with hormone-dependent forms of the disease (luminal carcinomas). However, the available data show that pregnancy in women in remission after treatment for breast cancer is possible and safe, even with the use of assisted reproductive techniques.

Preservation of fertility in young patients with breast cancer, including BRCA1, BRCA2 mutation carriersMain topic

Martina Zimovjanová

Onkologie. 2023:17(3):164-168 | DOI: 10.36290/xon.2023.033  

Preservation of fertility in cancer patients, also known as oncofertility, is a relatively new trend. For breast cancer patients with unfulfilled reproductive plans, the possibility of starting a family is a fundamental parameter of quality of life after overcoming cancer. This review aims to summarize current information on fertility preservation options for young patients. Available and effective options for preserving fertility include cryopreservation of oocytes/embryos or cryopreservation of ovarian tissue. When evaluating individual strategies, it is necessary to carefully consider the patient's characteristics, her disease, and the type of treatment....

Sacituzumab govitecan in the treatment of metastatic triple negative breast cancerMain topic

Markéta Palácová

Onkologie. 2023:17(3):160-163 | DOI: 10.36290/xon.2023.032  

Triple negative breast cancer (TNBC) continues to have the worst prognosis compared to other phenotypes. However, in the last 2 years it has been shining on better times. In addition to chemotherapy, new treatment options for metastatic disease - immunotherapy, PARP inhibitors and monoclonal antibody conjugate with cytostatic - sacituzumab govitekan have entered clinical practice. This drug demonstrated effectiveness not only in the progression free survival, but also in overall survival. Sacituzumab govitekan was introduced into clinical practice by the ASCENT registration study. Adverse events should be monitored for the most frequent occurrence...

Treatment strategy of triple negative breast cancer in young women - do we treat them differently?Main topic

Zuzana Bielčiková

Onkologie. 2023:17(3):157-159 | DOI: 10.36290/xon.2023.031  

Breast cancer in young women (YBC) is often associated with aggressive histology and a higher stage of the disease at the time of diagnosis. Triple-negative breast cancer (TNBC), the presence of a hereditary mutations, concerns about chemotherapy-induced amenorrhoea and/or concerns about prognosis are key words in the treatment of patients with YBC. However, current data have not shown a superiority of a particular type of systemic treatment or local methods of therapy tied to a population of patients under 40 years of age with TNBC. Also, the prognosis of TNBC in patients with YBC does not differ significantly from that of older women with TNBC. This...

Treatment issues of HER2 positive breast cancer in adult young patientsMain topic

Miloš Holánek

Onkologie. 2023:17(3):150-156 | DOI: 10.36290/xon.2023.030  

HER2-positive cancer is an aggressive subtype of breast tumors and requires complex oncological treatment. In patients with early-stage disease, the goal is to achieve long-term remission of the disease, while in patients with metastatic disease, the goal of treatment is to achieve long-term disease control, prolong overall survival and influence disease symptoms to improve quality of life. The treatment of adult young breast cancer patients is specific in many ways. This subset of patients is more likely to be diagnosed with aggressive subtypes, more advanced diseases, and higher-grade tumors with lower steroid receptor expression. Hereditary carcinomas...