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Simple Summary: Sézary syndrome (SS) is a rare and aggressive form of cutaneous T-cell lymphoma. Despite various treatments, it remains incurable, with a median survival of only 2.1 years. This retrospective study of 17 SS patients in West Sweden from 2012 to 2024 aimed to understand demographic characteristics, treatment effectiveness, and disease progression. Only 35% of patients showed the classic symptoms at diagnosis, indicating the need for personalized diagnostic approaches. Different treatment modalities were used, but combination therapy showed advantages in median survival over monotherapies. Notably, triple therapy involving retinoids, interferon alpha, and extracorporeal photopheresis (ECP) exhibited the longest median time to the next treatment, at 14.1 months. However, early initiation of ECP did not improve outcomes. This study underscores the complexity of SS, emphasizes the urgent need for more effective treatments, and highlights the importance of future prospective research in optimizing treatment strategies. Sézary syndrome (SS) is a rare primary cutaneous T-cell lymphoma variant. Despite various treatment options, it remains incurable, with a poor prognosis. There is an urgent need for additional descriptive research to enhance our understanding and treatment of SS. The aim of this retrospective register-based study was to outline patients' demographic characteristics; investigate the clinical, histopathological, and molecular findings; and assess treatment effectiveness with a focus on time to next treatment (TTNT) and disease progression. Data on 17 patients with SS were obtained from the primary cutaneous lymphoma register in West Sweden between 2012 and 2024. The results revealed that not all patients exhibited the classical triad of symptoms at diagnosis, emphasizing the need for personalized diagnostic approaches. The median survival was only 2.1 years, which reflects the aggressive nature of SS. The longest median TTNT was observed in triple therapy involving retinoids, interferon alpha, and extracorporeal photopheresis (ECP). There was no significant difference in TTNT between various lines of treatment. Early initiation of ECP treatment did not result in improved outcomes. This study highlights the importance of combination therapy for improved outcomes and underscores the need for future studies to identify optimal treatment approaches. [ABSTRACT FROM AUTHOR]

Simple Summary: Cancers of the biliary tract are rare but severe with high mortality rates. Randomised controlled trials suggest that chemotherapies such as gemcitabine and oxaliplatin (GemOx) may relieve symptoms and prolong life, but less is known on the efficacy and safety of such regimens in real life. The current paper assessed the real-world outcome of GemOx in all patients with advanced biliary tract cancer treated at any cancer centre in the South East Region of Sweden over a period of nine years. The median overall survival was nine months and time to disease progression five months. Prognostic factors such as performance status and gall bladder (rather than bile duct) localisation of the primary tumour were identified. Most patients received a lower dose of oxaliplatin than proposed by previous studies, which seemed feasible as few patients had severe adverse events. This study supports further use of GemOx as standard of care. Background: Gemcitabine and oxaliplatin (GemOx) is a standard combination regimen in advanced biliary tract cancer (BTC). There is limited evidence on its efficacy and safety in real life. Methods: A retrospective multicentre cohort study in the South East Region of Sweden, covering nine years (2011–2020) and three hospitals where GemOx was treatment of choice, was designed. Clinicopathological prognostic parameters were explored. Results: One hundred and twenty-one patients with advanced BTC were identified. Median overall and progression-free survival (OS and PFS) were 8.9 (95% CI = 7.2–10.6) and 5.3 (95% CI = 3.8–6.7) months. Performance status according to Eastern Cooperative Oncology Group (PS according to ECOG) 1–2 and primary gallbladder carcinoma were independent predictors for poor OS. PS and derived neutrophil/lymphocyte ratio were predictive for PFS. The most common severe type of myelosuppresion was grade 3 neutropenia that was recorded in 8%. Fifty-three (43.8%) experienced at least one episode of unplanned hospitalisation. One hundred and seventeen (97%) received oxaliplatin with lower dosage than was utilized in previous phase III trials (80–85 vs. 100 mg/m2) and a majority received further dose reductions of oxaliplatin and/or gemcitabine. Conclusion: The outcome of GemOx in advanced BTC appears comparable in controlled trials and real-world contexts. A lower dose of oxaliplatin seems more tolerable without compromising the outcome. [ABSTRACT FROM AUTHOR]

Simple Summary: Mycosis fungoides (MF) is a rare and incurable disease, but there are a wide variety of treatment options. Since the condition is rare, only a few studies have been performed on this topic in Sweden. This study contributes to the knowledge of the epidemiological and clinical features and diagnostic findings in addition to the treatment modalities and responses in patients with diagnosed MF and/or followed up for a long period of time in Sweden. The results of this study can be used to improve clinical practice and stimulate future research. (1) Background: Mycosis fungoides (MF) is a variant of primary cutaneous T-cell lymphoma. The aim of this study was to describe the clinical features and epidemiological and diagnostic findings in addition to the treatment modalities and responses in patients with MF. Furthermore, comparisons between patients in the early stage and the advanced stage were evaluated. (2) Methods: A retrospective register-based study based on data collected from the primary cutaneous lymphoma register and medical records was performed at the Department of Dermatology and Venerology at Sahlgrenska University Hospital, Gothenburg, Sweden. (3) Results: Eighty-four patients with a median age of 55 years with MF were included. Most of the patients (n = 73) were diagnosed at the early stage of the disease (IA–IIA). Overall disease progression was seen in 12.5% (n = 9) of the patients. Nine (10.7%) patients were deceased, out of which four (4.8%) deaths were associated with MF-related causes. (4) Conclusions: This study contributes to the knowledge of the epidemiological and clinical features in addition to the diagnostic findings and treatment responses in patients with MF in Sweden. [ABSTRACT FROM AUTHOR]

Simple Summary: Familial risk of cancer implies that two or more family members are diagnosed with the same cancer. This may be due to the genes or environmental factors that family members share. Familial risk for liver and gallbladder cancer is about 2.7 which means that when one family member is diagnosed with these cancers other family members have 2.7 times higher risk of being diagnosed with the same cancers compared to families were no member is yet diagnosed with these cancers. Risk between spouses is entirely due to shared environmental factors and for liver cancer there is a small risk. The most important way to prevent these cancers is to avoid their risk factors, alcohol, smoking and overweight, and to seek medical care for diabetes and liver infections. We used the Swedish Cancer Registry data to address familial risks for concordant (same) and discordant (different) hepatobiliary cancers, including their associations with any other cancers and with known risk factors. Risks were also assessed between spouses. The analysis covered Swedish families and their cancers between years 1958 and 2018. Adjusted familial risks were expressed as standardized incidence ratios (SIRs). Familial SIRs for concordant hepatocellular carcinoma (HCC) were 2.60, and for gallbladder cancer they were at the same level (2.76). Familial risk was also found for intrahepatic bile duct cancer and for female extrahepatic bile duct cancer. HCC was associated with lung and cervical cancers; extrahepatic bile duct and ampullary cancers were associated with colon and pancreatic cancers, suggesting Lynch syndrome. Among spouses, hepatobiliary cancer was associated with HCC, stomach, pancreatic, cervical and upper aerodigestive tract cancers. Among risk factors, family members diagnosed with alcohol-related disease showed association with HCC. The observed familial risks for hepatobiliary cancers were relatively high, and considering the poor prognosis of these cancers, prevention is of the utmost importance and should focus on moderation of alcohol consumption, vaccination/treatment of hepatitis viral infections and avoidance of overweight and other risk factors of type 2 diabetes. [ABSTRACT FROM AUTHOR]

Simple Summary: Individuals who are born with a disease-causing variant of the TP53 gene (hereditary TP53-related cancer syndrome, hTP53rc), also known as the Li–Fraumeni syndrome, have a very high (70–100%) lifetime risk of developing cancer and at younger ages. Carriers are also prone to develop secondary tumours due to irradiation. Current guidelines recommend surveillance programmes within studies and the use of non-irradiation modalities such as whole-body MRI (WB-MRI). In 2016, the Swedish TP53 study (SWEP53) started inclusion, offering a surveillance program including WB-MRI. With this study, we aimed to describe the rate, anatomical distribution of malignant, indeterminate, and benign imaging findings as well as the associated further workup generated by the baseline WB-MRI in adult study participants. Our study identified the need of further workup in 19/61 participants, of whom three patients had a new cancer. WB-MRI appears to be a valuable surveillance strategy in families with hTP53rc syndrome. A surveillance strategy of the heritable TP53-related cancer syndrome (hTP53rc), commonly referred to as the Li–Fraumeni syndrome (LFS), is studied in a prospective observational nationwide multi-centre study in Sweden (SWEP53). The aim of this sub-study is to evaluate whole-body MRI (WB-MRI) regarding the rate of malignant, indeterminate, and benign imaging findings and the associated further workup generated by the baseline examination. Individuals with hTP53rc were enrolled in a surveillance program including annual whole-body MRI (WB-MRI), brain-MRI, and in female carriers, dedicated breast MRI. A total of 68 adults ≥18 years old have been enrolled to date. Of these, 61 fulfilled the inclusion criteria for the baseline MRI scan. In total, 42 showed a normal scan, while 19 (31%) needed further workup, of whom three individuals (3/19 = 16%) were diagnosed with asymptomatic malignant tumours (thyroid cancer, disseminated upper GI cancer, and liver metastasis from a previous breast cancer). Forty-three participants were women, of whom 21 had performed risk-reducing mastectomy prior to inclusion. The remaining were monitored with breast MRI, and no breast tumours were detected on baseline MRI. WB-MRI has the potential to identify asymptomatic tumours in individuals with hTP53rc syndrome. The challenge is to adequately and efficiently investigate all indeterminate findings. Thus, a multidisciplinary team should be considered in surveillance programs for individuals with hTP53rc syndrome. [ABSTRACT FROM AUTHOR]

Simple Summary: Psychological resilience is an important psychological mechanism that enables a person to successfully handle significant adversities, e.g., a cancer diagnosis. Despite improved prognosis, breast cancer is associated with emotional distress across the trajectory of the disease. This study aimed to investigate psychological resilience and health-related quality of life in Swedish women with breast cancer at diagnosis and one year later. The resilience score declined in the cohort and was associated with health-related quality of life at both time points. Assessment of psychological resilience in breast cancer care might enable the identification of patients in need of intensified rehabilitation to improve their health-related quality of life. Psychological resilience is considered a major protective psychological mechanism that enables a person to successfully handle significant adversities, e.g., a cancer diagnosis. Higher levels of resilience have been associated with higher levels of health-related quality of life (HRQoL) in breast cancer (BC) patients, but research examining the longitudinal process of resilience is limited. The aim of this population-based longitudinal study was to investigate resilience and HRQoL from diagnosis to one year later in 418 Swedish women with primary BC. Resilience was measured with the Connor–Davidson Resilience Scale 25, and HRQoL was measured with the Short Form Health Survey. The participants responded to questions regarding demographic and study-specific variables. Clinicopathological variables were collected from the Swedish National Quality Register for Breast Cancer. The mean score for resilience was 70.6 (standard deviation, SD = 13.0) at diagnosis and 68.9 (SD = 14.0) one year later, p < 0.001. The level of trust in the treatment and financial situation demonstrated the greatest association with the change in resilience levels. No oncological treatment modality was associated with a change in resilience levels. HRQoL decreased over time in the cohort. Resilience was positively associated with HRQoL at one year post diagnosis, which demonstrates that resilience is an important factor in maintaining HRQoL. [ABSTRACT FROM AUTHOR]

Simple Summary: Real-world data (RWD), i.e., data reflecting normal clinical practice collected outside the constraints of randomised controlled trials, provide important insights into our understanding of prostate cancer and its management. Clinical cancer registries are an important source of RWD. Depending on their scope and the potential linkage to other data sources, registry-based data can be utilised to address a variety of questions including risk factors, healthcare utilisation, treatment effectiveness, adverse effects, disparities in healthcare access, quality of care and healthcare economics. This review describes the various registry-based RWD sources for prostate cancer research in Sweden (namely the National Prostate Cancer Register, the Prostate Cancer data Base Sweden (PCBaSe) and the Patient-overview Prostate Cancer) and documents their utility for better understanding prostate cancer aetiology and improving clinical care. Real-world data (RWD), that is, data from sources other than controlled clinical trials, play an increasingly important role in medical research. The development of quality clinical registers, increasing access to administrative data sources, growing computing power and data linkage capacities have contributed to greater availability of RWD. Evidence derived from RWD increases our understanding of prostate cancer (PCa) aetiology, natural history and effective management. While randomised controlled trials offer the best level of evidence for establishing the efficacy of medical interventions and making causal inferences, studies using RWD offer complementary evidence about the effectiveness, long-term outcomes and safety of interventions in real-world settings. RWD provide the only means of addressing questions about risk factors and exposures that cannot be "controlled", or when assessing rare outcomes. This review provides examples of the value of RWD for generating evidence about PCa, focusing on studies using data from a quality clinical register, namely the National Prostate Cancer Register (NPCR) Sweden, with longitudinal data on advanced PCa in Patient-overview Prostate Cancer (PPC) and data linkages to other sources in Prostate Cancer data Base Sweden (PCBaSe). [ABSTRACT FROM AUTHOR]