Portuguese Pancreatic Club Perspectives on Pancreatic Neuroendocrine Neoplasms: Diagnosis and Staging, Associated Genetic Syndromes and Particularities of Their Clinical Approach.

Pancreatic neuroendocrine neoplasms (panNENs) have been historically regarded as rare, but their incidence has raised more than 6-fold over the last 3 decades, mostly owing to improvement in the detection of small asymptomatic tumours with imaging. Early detection and proper classification and staging are essential for the prognosis and management of panNENs. Histological evaluation is mandatory in all patients for the diagnosis of panNEN. Regarding localization and staging, multiphasic contrast-enhanced computer tomography is considered the imaging study of choice. Nevertheless, several other diagnostic modalities might present complementary information that can help in diagnosis and staging optimization: magnetic resonance imaging, somatostatin receptor imaging using positron emission tomography in combination with computed tomography (PET/CT), PET/CT with fluorodeoxyglucose (18F-FDG), and endoscopic ultrasound. Approximately 10% of panNENs are due to an inherited syndrome, which includes multiple endocrine neoplasia type 1, von Hippel-Lindau disease, neurofibromatosis type 1 (NF-1), tuberous sclerosis complex, and Mahvash disease. In this review, the Portuguese Pancreatic Club summarizes the classification, diagnosis, and staging of panNENs, with a focus on imaging studies. It also summarizes the characteristics and particularities of panNENs associated with inherited syndromes.

As neoplasias neuroendócrinas pancreáticas (panNENs) são historicamente consideradas raras, embora a sua incidência tenha aumentado mais de 6 vezes nas últimas três décadas, principalmente devido à otimização do diagnóstico de tumores pequenos e assintomáticos em exames de imagem. A deteção precoce, a classificação e o estadiamento adequados são essenciais para o prognóstico e abordagem dos panNENs. A avaliação histológica é obrigatória em todos os doentes para o diagnóstico de panNENs. Para a localização e estadiamento, ​​a TC multifásica com contraste é considerada o estudo de imagem de eleição. Contudo, várias outras modalidades diagnósticas podem apresentar informações complementares que podem auxiliar no diagnóstico e na otimização do estadiamento: ressonância magnética, PET/CT dos receptores da somatostatina, PET/CT [18F]FDG e ecoendoscopia. Aproximadamente 10% dos panNENs estão relacionados com síndromes hereditários, que incluem neoplasia endócrina múltipla tipo 1 (MEN1), doença de von Hippel-Lindau (VHL), neurofibromatose tipo 1 (NF1), complexo de esclerose tuberosa (TSC) e doença de Mahvash. Neste artigo, o Clube Português de Pâncreas aborda a classificação, diagnóstico e estadiamento de panNENs, ​​com foco nos estudos de imagem, bem como resume as características e particularidades dos panNENs associados aos síndromes hereditários.

Intraoperative and immediate postoperative transfusion: Clinical-hematological profile of transfunded patients in a university hospital.

INTRODUCTION: The transfusion practice by surgery blood reserve, varied among services, must be performed through the rational and restrictive use of blood components because it is a scarce and expensive resource for health care services. OBJECTIVE: Analyze the use of blood products for surgery blood reserve by means of the study of the clinical-hematological profile of patients submitted to intraoperative and immediate postoperative transfusions. METHODS: This was an observational, cross-sectional, and retrospective study, conducted by collecting biological, operational, and laboratory variables, involving 680 patients at a university hospital who had elective surgery with surgery blood reserve request sent during the period from October 2021 to October 2022. RESULTS: The overall transfusion rate was 25.44%, and the mean preoperative hemoglobin level of transfused patients was 9.74 ± 2.50 g/dL, with the mean number of transfusions packed red blood cell units was 1.58 ± 0.77. Patients with higher preoperative hemoglobin levels were less likely to have transfusion (p < 0.001) and patients who had surgical oncologic were more likely to require transfusion (p = 0.048). The transfusion rate of packed red blood cells and platelets concentrates, compared to what was requested, was 15.86% and 5.82%. CONCLUSION: There is a tendency of transfusions to follow restrictive models, with higher transfusion probability in surgical oncologic. Furthermore, there should be more a conscise use of the surgery blood reserves request.

Acanthosis Nigricans Manifesting as a Paraneoplastic Syndrome Associated With Cholangiocarcinoma.

We present the case of a 64-year-old woman with type 2 diabetes who was diagnosed with early-stage intrahepatic cholangiocarcinoma and underwent partial hepatectomy followed by adjuvant chemotherapy. The patient simultaneously developed skin lesions compatible with acanthosis nigricans (AN). Thirty-seven months after completing chemotherapy, the patient had a recurrence of extensive skin and mucosal lesions compatible with AN. A thoracic-abdominal-pelvic (TAP) CT showed a relapse with hepatic hilar adenopathy. Currently, she is under evaluation to undergo radical treatment. Malignancy is a rare cause of AN and skin lesions can arise before, during, or after the diagnosis. As a paraneoplastic syndrome, it is usually related to gastric adenocarcinoma, with cholangiocarcinoma being a rare entity in this setting. Although an uncommon manifestation, the malignant etiology should be considered among other prevalent causes, such as metabolic disorders, and establishing an association can lead to an early diagnosis and initiation of curative treatment.

Prognostic Value of Bladder Involvement in the Outcome of Upper Tract Urothelial Carcinoma.

Accurately predicting the clinical prognosis of upper tract urothelial carcinoma (UTUC) seems crucial. We evaluated the effect of the involvement of urothelial bladder carcinoma (UBC) as a potential prognostic factor for overall survival (OS) and progression-free survival (PFS). The cohort included 115 patients with UTUC, subgrouped between January 2009 and December 2019 as follows: (1) only UTUC and (2) UTUC with synchronous or metachronous UBC (UTUC + UBC). Univariate and multivariate analyses were performed to identify independent prognostic factors for OS and PFS. Synchronous or metachronous UBC diagnosis in UTUC patients was an independent predictor of worse PFS (HR 3.326 CI 95% 1.474−7.503, p = 0.004), but it was not identified as a prognostic factor for OS (p > 0.05). Lymphovascular invasion (LVI) was associated with decreased PFS (HR 2.687 CI 95%1.172−6.163, p = 0.020) and OS (HR 4.980 CI 95%1.763−14.064, p = 0.002). This study indicates that concomitant or later UBC could predict a poor PFS, but it is not associated with a significantly worse OS in UTUC patients. The prognostic impact of LVI underlines its inclusion in the tumor staging system of UTUC.

Late Recurrence of Pancreatic Solid Pseudopapillary Neoplasm With Peritoneal Carcinomatosis Treated With Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC): A Case Report.

Pancreatic solid pseudopapillary neoplasm (SPN) is a rare malignant tumour predominantly affecting young women. The occurrence of peritoneal carcinomatosis (PC) in this setting is an even rarer condition, usually related to perioperative tumour rupture. We present a case of a 43-year-old woman who previously underwent distal splenopancreatectomy after the diagnosis of a pancreatic SPN. Thirteen years later, the patient underwent a radical hysterectomy due to a uterine myoma. Intraoperatively, a peritoneal mass was additionally found and resected. Histological examination revealed an implant with morphology compatible with pancreatic SPN. The patient was then referred to our institution. Staging MRI and CT revealed multiple nodular lesions adjacent to the left colon, suggestive of peritoneal implants. The patient was then submitted to cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) with oxaliplatin and irinotecan. Histological examination confirmed peritoneal involvement by a pancreatic SPN. The postoperative course was unremarkable. Two years after surgery, the patient remains asymptomatic with no evidence of relapse. Despite SPN being cancer with a relatively indolent evolution, one needs to be aware of a possible recurrence several years after the primary resection, mainly in patients with evidence of intraoperative tumour rupture.

Quality-of-life reporting in randomized controlled trials shaping the EAU Renal Cell Carcinoma Guidelines.

INTRODUCTION: Quality-of-life (QoL) metrics' importance in patient care has been increasingly recognized and has led oncological societies to issue clinical guidances on their collection. We aim to describe how current RCTs shaping the EAU RCC Guidelines collect QoL metrics and how long they report them. MATERIALS AND METHODS: We searched EAU Renal Cell Carcinoma Guidelines' references for phase 3 clinical trials. Data related to if, for how long and how QoL metrics were collected and/or reported were obtained. RESULTS: Over 35 identified trials, with 16 (46 %) having complete information, being included on our study. Among the analyzed studies, 11 (69 %) were on metastasized/advanced disease setting and 5 (31 %) in adjuvant therapy after surgery; none included QoL as a primary outcome, and 13 (81 %) reported positive QoL results. Regarding timings, all (100 %) studies reported QoL during intervention, and at end of treatment, 9 (56 %) until progression and none (0 %) until death. Median OS was reached in 11 (69 %) studies. Across all studies, the median QoL reporting time was of 7.6 (5.0-12.0) months and the median observation time of 27.1 (22.5-53.1) months. As such, trials reported QoL metrics for a of 28.0 % of the possible time at time of their publications. CONCLUSIONS: We found that only 46 % of RCTs reported on QoL metrics and most of these evaluated QoL during an insufficient timeframe. Most studies in this field are not adequately informing on QoL metrics, even when they are reported.

Metastatic Castration-Resistant Prostate Cancer with BRCA2 Mutation: The Challenge Incorporating PARP Inhibitors and Platinum in Treatment Sequencing.

Prostate cancer is the second most frequent malignancy in men worldwide. Despite the improvement in survival achieved by increasingly early diagnosis and advances in treatment, it is still associated with high mortality. Because of its molecular heterogeneity, there is a need to identify genetic alterations in order to apply targeted therapies. Increasing evidence suggests that the PARP inhibitor olaparib could have a significant synthetic lethal effect in prostate cancer with homologous recombination defects, such as BRCA1/2 mutations. It is not yet known if, under these circumstances, platinum-based chemotherapy induces higher response rates in prostate cancer. We present the case of a patient with BRCA2-mutated metastatic castration-resistant prostate cancer whose treatment sequence included carboplatin and olaparib. LEARNING POINTS: Metastatic castration-resistant prostate cancer (mCRPC) remains a lethal disease despite significant progress in treatment.The BRCA2 mutation is associated with worse survival and so timely genetic screening is important.Studies are needed to identify the best therapeutic sequencing strategy for mCRPC harbouring homologous recombination repair defects, which includes PARP inhibitors and platinum.

Reviewing Treatment Options for Advanced Renal Cell Carcinoma: Is There Still a Place for Tyrosine Kinase Inhibitor (TKI) Monotherapy?

Renal cell carcinoma (RCC) comprises a highly heterogeneous group of kidney tumours built upon distinct genetic- and epigenetic-driven mechanisms and molecular pathways. Therefore, responsiveness to treatment is considerably variable across patients, adding an extra layer of complexity to the already challenging therapeutic decision process. The last decade brought an unprecedented shift in the medical approach to advanced or metastatic RCC; in fact, immunotherapy-based combinations have significantly transformed the therapeutic arsenal and clinical outcomes of these patients. These strategies were quickly adopted by international guidelines committees as the new standards of care. However, this enhanced efficacy comes at the expense of tolerability, with a predictable negative impact on patients' quality of life. Moreover, subgroup and post hoc analyses of the major clinical trials have shown that not all patients benefit equally from these innovative approaches. In this context, a group of experts on kidney cancer met and discussed the state of the art in the field, with a special emphasis on the appropriateness of using monotherapy with an anti-angiogenesis tyrosine kinase inhibitor (TKI) to treat specific subgroups of patients with RCC. This article reviews the main topics that were considered to be pertinent for that discussion and establishes the profile of patients for whom TKI monotherapy remains a sensible frontline option by avoiding overtreatment and an unnecessary exposure to treatment-related toxicity.

Biomarkers for Bladder Cancer Diagnosis and Surveillance: A Comprehensive Review.

Bladder cancer (BC) ranks as the sixth most prevalent cancer in the world, with a steady rise in its incidence and prevalence, and is accompanied by a high morbidity and mortality. BC is a complex disease with several molecular and pathological pathways, thus reflecting different behaviors depending on the clinical staging of the tumor and molecular type. Diagnosis and monitoring of BC is mainly performed by invasive tests, namely periodic cystoscopies; this procedure, although a reliable method, is highly uncomfortable for the patient and it is not exempt of comorbidities. Currently, there is no formal indication for the use of molecular biomarkers in clinical practice, even though there are several tests available. There is an imperative need for a clinical non-invasive testing for early detection, disease monitoring, and treatment response in BC. In this review, we aim to assess and compare different tests based on molecular biomarkers and evaluate their potential role as new molecules for bladder cancer diagnosis, follow-up, and treatment response monitoring.

Metastatic castration-resistant prostate cancer and the challenge of a patient with chronic kidney disease in hemodialysis

At a time when the population shows increasing longevity, entities such as cancer and chronic kidney disease (CKD) are more frequently connected. In the United States, approximately 6% of the patients on hemodialysis have cancer. The challenge to manage oncologic patients with CKD in a hemodialytic program represents a great shortage of available information on the choice of the best drug, timing, dosage adjustments, dialysis method, and treatment safety. We present the case of a patient with prostate cancer and terminal CKD in hemodialysis, and the treatment sequence after the development of resistance to hormonal blockade therapy, which included docetaxel, enzalutamide, and radium-223.

Metastatic castration-resistant prostate cancer and the challenge of a patient with chronic kidney disease in hemodialysis.

At a time when the population shows increasing longevity, entities such as cancer and chronic kidney disease (CKD) are more frequently connected. In the United States, approximately 6% of the patients on hemodialysis have cancer. The challenge to manage oncologic patients with CKD in a hemodialytic program represents a great shortage of available information on the choice of the best drug, timing, dosage adjustments, dialysis method, and treatment safety. We present the case of a patient with prostate cancer and terminal CKD in hemodialysis, and the treatment sequence after the development of resistance to hormonal blockade therapy, which included docetaxel, enzalutamide, and radium-223.