BACKGROUND/AIM: Adenoid cystic carcinoma (AdCC) is a rare malignant tumor that primarily affects the salivary glands but can also occur in other organs. Low incidence and unpredictable clinical behavior make AdCC one of the most difficult head and neck tumors to treat. CASE REPORT: We present the case of a 54-year-old woman with AdCC localized at the base of the tongue, following radical surgical and oncological therapy. Due to advances in palliative oncological treatment, there is a more than five-year survival period before the progression of metastatic disease. Considering the rare occurrence of this disease, a literature search was also conducted, and therapy options are discussed. Ensuring a sufficient extent of the surgical procedure is still a challenge, and most specialists agree that subsequent postoperative radiotherapy reduces the risk of local recurrence. The effective dose of radiotherapy to the area of the primary tumor and lymph nodes is not clearly defined. CONCLUSION: The distinct biological behavior of AdCC results in varying sensitivity to chemotherapy or radiotherapy compared to treatments commonly used for head and neck squamous cell carcinomas. Treatment recommendations for these rarer tumors are based mainly on case reports and small clinical trials. The acquired therapeutic experience can contribute to prolonging the survival period of patients and improving their prognosis and quality of life.

• Klíčová slova
• Adenoid cystic carcinoma, breast cancer, duplicate cancer, head and neck cancer, metastatic disease,
• MeSH
• adenoidně cystický karcinom * terapie   diagnóza   patologie   sekundární   MeSH
• kombinovaná terapie MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory jazyka * patologie   terapie   diagnóza   MeSH
• nádory prsu * patologie   terapie   diagnóza   MeSH
• staging nádorů * MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

PURPOSE: The study aims to provide an updated overview of studies that show the impact of psychotherapeutic interventions on fear of cancer recurrence (FCR), to explore the relationship between FCR and related factors (psychological distress, well-being, anxiety, depression, fear, coping strategies, quality of life), and to identify the most effective therapeutic approaches in managing FCR. METHODS: Three databases were searched between January 2021 and April 2021 using the key words "fear of cancer recurrence - psychotherapy - intervention" following the a priori established PRISMA protocol. RESULTS: Thirteen studies were included in the final review. The database search identified 239 potential papers. After removing duplicates and irrelevant articles by title and language, population, and type of study, 13 articles were assessed for eligibility of the abstract, and 13 full-text articles were reviewed and included in this systematic review. The studies were mainly from the Netherlands (4 out of 13). Positive benefits of therapeutic interventions on FCR were reported. CONCLUSIONS: This research highlights challenges in using therapeutic approaches in dealing with FCR and its management. Therapeutic intervention is an effective means of managing not only FCR but also related factors (distress, well-being, quality of life). However, individual needs and preferences must be taken into consideration whilst choosing a therapeutic approach. Cognitive behavioural therapy (CBT), acceptance and commitment therapy (ACT), and mindfulness-based interventions are the most used approaches with CBT being the most effective, especially in a blended form (i.e. standard CBT combined with other self-help activities). IMPLICATIONS FOR CANCER SURVIVORS: The aim was to provide information about the most effective therapeutic approaches for coping with FCR.

• Klíčová slova
• Cancer, Fear of cancer recurrence, Intervention, Oncology, Psycho-oncology, Systematic review, Therapy,
• MeSH
• dospělí MeSH
• kvalita života MeSH
• lidé MeSH
• lokální recidiva nádoru terapie   psychologie   MeSH
• přežívající onkologičtí pacienti * psychologie   MeSH
• strach psychologie   MeSH
• terapie přijetí a odevzdání * MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• systematický přehled MeSH

• MeSH
• dítě MeSH
• fibrosarkom etiologie   MeSH
• kojenec MeSH
• lidé MeSH
• nádory nosohltanu etiologie   MeSH
• nádory oka radioterapie   MeSH
• nádory vyvolané zářením * MeSH
• předškolní dítě MeSH
• radioterapie škodlivé účinky   MeSH
• retinoblastom radioterapie   MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH

BACKGROUND: Chronic pancreatitis is a known risk factor of pancreatic cancer (PDAC). A similar association has been suggested but not demonstrated for autoimmune pancreatitis (AIP). OBJECTIVE: The aim of our study was to identify and analyse all published cases of AIP and PDAC co-occurrence, focusing on the interval between the diagnoses and the cancer site within the pancreas. METHODS: Relevant studies were identified through automatic searches of the MEDLINE, EMBASE, Scopus, and Web of Science databases, and supplemented by manual checks of reference lists in all retrieved articles. Missing/unpublished data were obtained from the authors of relevant publications in the form of pre-prepared questionnaires. RESULTS: A total of 45 cases of PDAC in AIP patients were identified, of which 12 were excluded from the analysis due to suspicions of duplicity or lack of sufficient data. Thirty-one patients (94%) had type 1 AIP. Synchronous occurrence of PDAC and AIP was reported in 11 patients (33%), metachronous in 22 patients (67%). In the metachronous group, the median period between diagnoses was 66.5 months (2-186) and a majority of cancers (86%) occurred more than two years after AIP diagnosis. In most patients (70%), the cancer originated in the part of the pancreas affected by AIP. CONCLUSIONS: In the literature, there are reports on numerous cases of PDAC in AIP patients. PDAC is more frequent in AIP type 1 patients, typically metachronous in character, and generally found in the part of the pancreas affected by AIP.

• Klíčová slova
• Autoimmune pancreatitis, Chronic pancreatitis, Immunoglobulin G4-Related disease, Malignancy, Pancreatic cancer,
• MeSH
• autoimunitní nemoci * komplikace   diagnóza   epidemiologie   MeSH
• autoimunitní pankreatitida * MeSH
• diferenciální diagnóza MeSH
• lidé MeSH
• nádory slinivky břišní * komplikace   diagnóza   epidemiologie   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• scoping review MeSH

INTRODUCTION: The occurence of synchronous pancreatic cancer and other primary cancer is not frequent and reaches about 5.6% as reported in autoptic studies. Double resections of the pancreas with another organ due to synchronous malignancies have been published only in quite sporadic sets of cases or individual case reports. The authors present three cases of synchronous pancreatic malignancies and stomach or renal cancers treated with surgery, including results and survival. CASES: Three patients with synchronous double cancer were identified in a series of 400 pancreatic resections (20062014). Two patients presented with symptoms of pancreatic periampullary tumors (bile duct obstruction, weight loss and abdominal pain). The second malignancies were identified as incidental during diagnostic work-up (asymptomatic cancer of the stomach, kidney). Pancreatoduodenectomies (PDE) with lymphadenectomies were performed due to ductal adenocarcinomas (pT2N1M0 G3 and pT3N1M0 G2). The second procedures included subtotal gastrectomy with lymphadenectomy (gastric adenocarcinoma pT1N1M0, G2) and nephrectomy (renal papillary carcinoma pT1bN0M0, G3). Postoperative adjuvant chemotherapy with gemcitabine was given in both patients. Survival rates were 12 and 19 months, respectively. The third patient suffered from abdominal pain and weight loss. Diagnostic work-up revealed stomach carcinoma and early pancreatic adenocarcinoma. Double resection - subtotal gastrectomy with lymphadenectomy and pancreatoduodenectomy with lymphadenectomy - was performed. Gastric adenocarcinoma pT2N2M0, G3 and pancreatic ductal papillary-mucinous adenocarcinoma pT2N0M0, G1 were found in the specimens. Adjuvant radiochemotherapy with 5-fluorouracil and leukovorine was given postoperatively. This patient is still alive nearly 5 years after the surgery, without any reccurence. CONCLUSION: The survival of patients with double synchronous pancreatic malignancies and other primary tumors in our set seems to be influenced by the stage and biology of pancreatic cancer. The survival was worse when the duplicity was presented with symptoms of pancreatic cancer. Pancreatic cancer found incidentally when another malignancy is presented has more favourable results.

• MeSH
• adenokarcinom chirurgie   MeSH
• duktální karcinom slinivky břišní chirurgie   MeSH
• gastrektomie metody   MeSH
• karcinom z renálních buněk chirurgie   MeSH
• lidé MeSH
• mnohočetné primární nádory chirurgie   MeSH
• nádory ledvin chirurgie   MeSH
• nádory slinivky břišní chirurgie   MeSH
• nádory žaludku chirurgie   MeSH
• nefrektomie metody   MeSH
• pankreatektomie metody   MeSH
• pankreatoduodenektomie metody   MeSH
• senioři MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

BACKGROUND: Several reports indicate that inherited mutations in the PALB2 gene predispose to breast cancer. However, there is little agreement about the clinical relevance and usefulness of mutation screening in this gene. We analyzed the prevalence and spectrum of germline mutations in PALB2 to estimate their contribution to hereditary breast and/or ovarian cancer in the Czech Republic. METHODS: The entire PALB2 coding region was sequenced in 409 breast/ovarian cancer patients negative for BRCA1 and BRCA2 mutations. Testing for large genomic rearrangements (LGR) was performed by multiplex ligation-dependent probe amplification (MLPA) analysis. RESULTS: We have identified 13 different pathogenic alterations including 10 truncating mutations and three LGRs in 16 of 409 patients (3.9%), whereas one truncating mutation was found in a group of 1,226 controls (0.08%; P = 2.6 × 10(-9)). Three novel LGRs included deletions involving exons 7-8 and 9-10, respectively, and a duplication spanning exons 9-11. Five frameshift and two nonsense mutations were novel, whereas three truncating mutations were described previously. The only recurrent mutation was the c.172_175delTTGT detected in four unrelated breast cancer individuals. CONCLUSIONS: Our analyses demonstrated the significant role of the PALB2 gene in breast cancer susceptibility. The highest frequency of PALB2 mutations (comparable with that previously reported for BRCA2) was found in a subgroup of patients with hereditary breast cancer (HBC) (13/235; 5.5%). IMPACT: Our results show that mutation analysis of the PALB2 gene, including the analysis of LGRs, is primarily indicated in patients with HBC in case of their BRCA1 and BRCA2 negativity.

• MeSH
• delece genu MeSH
• genetická predispozice k nemoci MeSH
• geny BRCA1 * MeSH
• geny BRCA2 * MeSH
• jaderné proteiny genetika   MeSH
• lidé MeSH
• molekulární sekvence - údaje MeSH
• mutační analýza DNA metody   MeSH
• nádorové supresorové proteiny genetika   MeSH
• nádory prsu genetika   patologie   MeSH
• nádory vaječníků genetika   patologie   MeSH
• prevalence MeSH
• protein FANCN MeSH
• rizikové faktory MeSH
• rodokmen MeSH
• sekvence nukleotidů MeSH
• studie případů a kontrol MeSH
• Check Tag
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Názvy látek
• jaderné proteiny MeSH
• nádorové supresorové proteiny MeSH
• PALB2 protein, human MeSH Prohlížeč
• protein FANCN MeSH

Atopic dermatitis is a chronic inflammatory intensively pruritic skin disease. Patients with moderate-to-severe atopic dermatitis or with difficult-to-treat areas are candidates for systemic therapy, especially when topical therapy is inadequate. Currently, we have available not only conventional immunosuppressive systemic therapy, but also targeted biological therapy, which has shown a remarkable reduction in clinical severity with a good safety profile. Dupilumab has been approved to treat moderate-to-severe atopic dermatitis. Even though the therapy has been available for more than 3 years, there are still limited data regarding the treatment of patients with concomitant cancer. Previous immunosuppressive treatment for atopic dermatitis, such as cyclosporine or azathioprine, poses a safety risk for patients with malignant disease. We present a case series of three patients with advanced cancer and severe atopic dermatitis treated with dupilumab for an average of 17 months with a great response toward atopic dermatitis without cancer recurrence. One patient had colorectal cancer' the second and the third both had cancer duplicity-colorectal and kidney cancer and penile squamous cell carcinoma with prostate cancer. Our cases suggest that dupilumab can safely control atopic dermatitis in patients with advanced cancer.

• Klíčová slova
• atopic dermatitis, biological therapy, cancer, dupilumab, real-world study,
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

Accurate copying of DNA during S phase is essential for genome stability and cell viability. During genome duplication, the progression of the DNA replication machinery is challenged by limitations in nucleotide supply and physical barriers in the DNA template that include naturally occurring DNA lesions and secondary structures that are difficult to replicate. To ensure correct and complete replication of the genome, cells have evolved several mechanisms that protect DNA replication forks and thus maintain genome integrity and stability during S phase. One class of enzymes that have recently emerged as important in this process, and therefore as promising targets in anticancer therapy, are the poly(ADP-ribose) polymerases (PARPs). We review here the roles of these enzymes during DNA replication as well as their impact on genome stability and cellular viability in normal and cancer cells.

• Klíčová slova
• DNA replication stress, DNA strand break repair, Okazaki fragment, PARP1, PARP2, poly(ADP-ribose),
• MeSH
• aktivace enzymů MeSH
• cílená molekulární terapie MeSH
• lidé MeSH
• multigenová rodina MeSH
• náchylnost k nemoci MeSH
• nestabilita genomu MeSH
• oprava DNA MeSH
• PARP inhibitory farmakologie   terapeutické užití   MeSH
• poly(ADP-ribosa)polymerasy genetika   metabolismus   MeSH
• poškození DNA MeSH
• proliferace buněk MeSH
• protinádorové látky farmakologie   terapeutické užití   MeSH
• replikace DNA MeSH
• S fáze fyziologie   MeSH
• zvířata MeSH
• Check Tag
• lidé MeSH
• zvířata MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH
• Názvy látek
• PARP inhibitory MeSH
• poly(ADP-ribosa)polymerasy MeSH
• protinádorové látky MeSH

• MeSH
• lidé středního věku MeSH
• lidé MeSH
• mnohočetné primární nádory diagnóza   genetika   MeSH
• nádory průdušek diagnóza   genetika   MeSH
• nádory žaludku diagnóza   genetika   MeSH
• rodokmen MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH

OBJECTIVE: This article reviews the topic of hereditary ovarian cancer, describes persons at risk of hereditary disposition to cancer and gives instructions for genetic counselling and molecular analysis, including contacts to specialized centres in the Czech Republic. SUBJECT: Review. SETTING: Institute of Biochemistry and Experimental Oncology, Charles University in Prague. METHODS: Hereditary ovarian cancer occurs in three autosomal dominant syndromes: appropriate hereditary ovarian cancer (HOC), hereditary breast and ovarian cancer (HBOC) and hereditary non-poliposis colorectal cancer (HNPCC). Physician in practice or specialist at the clinic should focus interest on patients form families with frequent occurrence of breast and/or ovarian cancer, patients with early onset disease or tumour duplicity (breast and ovarian cancer). Hereditary disposition to ovarian (and breast) cancer could be assessed by molecular genetic analysis of two main susceptibility genes BRCA1 and BRCA2, or other genes in families with diverse tumours. Molecular genetic analysis should be in any cases indicated by experienced clinical genetic. In the Czech Republic, the consensus of genetic and clinical care of risk patients was published and specialized centres for families with hereditary predisposition were settled in Prague and Brno. CONCLUSION: Persons with hereditary susceptibility to cancer constitute noted group where painstaking dispensarisation and preventive care may prevent malignancy or detect it in the early stage.

• MeSH
• dědičné nádorové syndromy genetika   MeSH
• geny BRCA1 MeSH
• geny BRCA2 MeSH
• lidé MeSH
• mutace MeSH
• nádory prsu genetika   MeSH
• nádory vaječníků genetika   MeSH
• Check Tag
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• přehledy MeSH