Pleuropulmonary blastoma and DICER1-related tumor predisposition: from clinicopathologic observations to clinical trial.

Purpose Of Review: Pleuropulmonary blastoma (PPB) is a rare primary lung neoplasm of infancy and childhood. The purpose of this review is to highlight recent developments in our understanding of PPB and research strategies to facilitate future rare cancer research.

Recent Findings: The International PPB/DICER1 Registry has recently assembled the largest-ever cohorts of type I and Ir PPB and type II and III PPB.

Unravelling the natural history of localised prostate cancer in the post-prostate specific antigen era: implications for clinical management.

Background: Management of localised prostate cancer (PCa) remains controversial in the era of prostate-specific antigen (PSA) testing. This study aims to describe the natural history of men with PCa being followed up expectantly to evaluate disease mortality.

Methods: After exclusion, clinical data of 204 patients retrieved from a prospective large PCa registry were reviewed.

PD-L1 expression is mediated by microRNA processing, Wnt/β-catenin signaling, and chemotherapy in Wilms tumor.

Inhibition of immune checkpoint proteins is effective in adult cancers but has shown limited efficacy in pediatric cancers. While factors regulating expression of immune checkpoint proteins such as PD-L1 are well-documented in adult cancers, their regulation is poorly understood in pediatric cancers. Here, we show that PD-L1 is upregulated in distinct subsets of Wilms tumor, the most common pediatric kidney cancer.

Radiation-Induced Hemorrhagic Cystitis in Prostate Cancer Survivors: The Hidden Toll.

: Radiation therapy (RT) plays a crucial role in managing prostate cancer, offering effective disease control and improving survival rates in both localized and recurrent cases. However, RT can lead to hemorrhagic cystitis, a significant late complication resulting in chronic morbidity and other health issues. This study aims to evaluate the real-world incidence of radiation-induced hemorrhagic cystitis requiring surgical intervention.

Lost opportunities: the underutilization of castrate-resistant prostate cancer treatment in real-world settings.

Background: Various treatment regimens are now available for metastatic castrate-resistant prostate cancer (CRPC). This work evaluates the real-world prescription patterns of CRPC in a large tertiary care center and the factors influencing them.

Methods: Health records of 330 patients with metastatic hormone-sensitive prostate cancer (HSPC), treated and progressed to CRPC between 2016 and 2020, were reviewed from a prospective uro-oncological database.

The Association of Obstructive Sleep Apnea with Urological Cancer Incidence and Mortality-A Systematic Review and Meta-analysis.

Background And Objective: While obstructive sleep apnea (OSA) and urological cancer are both strongly associated with hypoxia, controversy exists regarding their association with each other. This study aims to summarize and synthesize evidence to clarify the association between OSA and urological cancer incidence and mortality.

Methods: According to a prespecified protocol, PubMed, Embase, Cochrane Library, and Scopus were searched from inception to November 16, 2023, for observational and randomized studies reporting the association of OSA with urological cancer incidence or mortality.

Fully Automated Measurement of Cobb Angles in Coronal Plane Spine Radiographs.

scoliosis is a three-dimensional structural deformity characterized by lateral and rotational curvature of the spine. The current gold-standard method to assess scoliosis is the measurement of lateral curvature of the spine using the Cobb angle in coronal plane radiographs. The interrater variability for Cobb angle measurements reaches up to 10°.

Actinomycin D and bortezomib disrupt protein homeostasis in Wilms tumor.

Wilms tumor is the most common kidney cancer in children, and diffusely anaplastic Wilms tumor is the most chemoresistant histological subtype. Here we explore how Wilms tumor cells evade the common chemotherapeutic drug actinomycin D, which inhibits ribosomal RNA biogenesis. Using ribosome profiling, protein arrays, and a genome-wide knockout screen, we describe how actinomycin D disrupts protein homeostasis and blocks cell cycle progression.

Cardio-oncology in advanced prostate cancer.

Treatment intensification with androgen deprivation therapy (ADT) and androgen receptor pathway inhibitors (ARPi) have led to improved survival in advanced prostate cancer. However, ADT is linked to significant cardiovascular toxicity, and ARPi also negatively impacts cardiovascular health. Together with a higher prevalence of baseline cardiovascular risk factors reported among prostate cancer survivors at diagnosis, there is a pressing need to prioritise and optimise cardiovascular health in this population.

Androgen Receptor Splice Variant 7 in Asian Patients With Metastatic Castration-Resistant Prostate Cancer.

Purpose: Androgen receptor splice variant 7 (ARV-7) is a resistance mechanism to hormonal therapy in metastatic castrate-resistant prostate cancer (mCRPC). It has been associated with poor outcomes. On progression to castrate resistance, ARV-7 positivity has been identified in global populations at an incidence of 17.

Tips and Tricks: Evolution of Orchidectomy.

Radical orchidectomy has been the standard surgery for testicular tumours. While a straightforward routine surgery, there are several finer points in the surgical technique and perioperative care that urologists should be familiar with. This mini-review discusses modifications to the conventional surgical approach such as organ-sparing surgery and the subinguinal approach, and practice points regarding prostheses and sperm banking that are pertinent to early management of a patient with a testicular tumour.

Clinical Consultation Guide: Nerve-sparing Techniques for Retroperitoneal Lymph Node Dissection in Testicular Cancer.

Reptroperitoneal lymph node dissection (RPLND) is associated with a risk of morbidity and ejaculatory dysfunction. Nerve-sparing RPLND shows promise in preserving ejaculation alongside oncological efficacy. Laparoscopic and robot-assisted modalities are feasible with good outcomes, highlighting the need for ongoing scientific research and refinement of surgical skills.

Anaplastic sarcoma of the kidney (DICER1-sarcoma of the kidney): A report from the International Pleuropulmonary Blastoma/DICER1 Registry.

Background: Anaplastic sarcoma of the kidney (ASK) is a DICER1-related neoplasm first identified as a distinctive tumor type through the evaluation of unusual cases of putative anaplastic Wilms tumors. Subsequent case reports identified the presence of biallelic DICER1 variants as well as progression from cystic nephroma, a benign DICER1-related neoplasm. Despite increasing recognition of ASK as a distinct entity, the optimal treatment remains unclear.

Clinical Practice Guide: Management of Chylous Ascites After Retroperitoneal Lymph Node Dissection.

Chylous ascites is a rare but challenging complication after retroperitoneal lymph node dissection. Conservative management is successful in most cases, with percutaneous lymphangiography reserved for refractory instances. Surgical interventions are associated with high failure rates.

"Seeing Is Believing": Additive Utility of Ga-PSMA-11 PET/CT in Prostate Cancer Diagnosis.

Widespread adoption of mpMRI has led to a decrease in the number of patients requiring prostate biopsies. Ga-PSMA-11 PET/CT has demonstrated added benefits in identifying csPCa. Integrating the use of these imaging techniques may hold promise for predicting the presence of csPCa without invasive biopsy.

An imbalance between proliferation and differentiation underlies the development of microRNA-defective pineoblastoma.

Mutations in the microRNA processing genes and drive several cancers that resemble embryonic progenitors. To understand how microRNAs regulate tumorigenesis, we ablated or in the developing pineal gland to emulate the pathogenesis of pineoblastoma, a brain tumor that resembles undifferentiated precursors of the pineal gland. Accordingly, these mice develop pineal tumors marked by loss of microRNAs, including the let-7/miR-98-5p family, and de-repression of microRNA target genes.

Outcomes in ovarian Sertoli-Leydig cell tumor: A report from the International Pleuropulmonary Blastoma/DICER1 and Ovarian and Testicular Stromal Tumor Registries.

Objective: Sertoli-Leydig cell tumors (SLCTs) are rare sex cord-stromal tumors, representing <0.5% of all ovarian tumors. We sought to describe prognostic factors, treatment and outcomes for individuals with ovarian SLCT.

DROSHA Regulates Mesenchymal Gene Expression in Wilms Tumor.

Wilms tumor, the most common pediatric kidney cancer, resembles embryonic renal progenitors. Currently, there are no ways to therapeutically target Wilms tumor driver mutations, such as in the microRNA processing gene DROSHA. In this study, we used a "multiomics" approach to define the effects of DROSHA mutation in Wilms tumor.

Management of advanced prostate cancer in the Asia-Pacific region: Summary of the Asia-Pacific Advanced Prostate Cancer Consensus Conference 2023.

Aim: The aim of the third Asia-Pacific Advanced Prostate Cancer Consensus Conference (APAC APCCC 2023) was to discuss the application in the Asia-Pacific (APAC) region of consensus statements from the 4th Advanced Prostate Cancer Consensus Conference (APCCC 2022).

Methods: The one-day meeting in July 2023 brought together 27 experts from 14 APAC countries. The meeting covered five topics: (1) Intermediate- and high-risk and locally advanced prostate cancer; (2) Management of newly diagnosed metastatic hormone-sensitive prostate cancer; (3) Management of non-metastatic castration-resistant prostate cancer; (4) Homologous recombination repair mutation testing; (5) Management of metastatic castration-resistant prostate cancer.

Specifications of the ACMG/AMP Variant Classification Guidelines for Germline Variant Curation.

Germline pathogenic variants in predispose individuals to develop a variety of benign and malignant tumors. Accurate variant curation and classification is essential for reliable diagnosis of -related tumor predisposition and identification of individuals who may benefit from surveillance. Since 2015, most labs have followed the American College of Medical Genetics and Genomics and the Association for Molecular Pathology (ACMG/AMP) sequence variant classification guidelines for germline variant curation.

Off-clamp vs on-clamp robot-assisted partial nephrectomy: a systematic review and meta-analysis.

Objective: To compare intra- and postoperative outcomes between off-clamp and on-clamp robot-assisted partial nephrectomy (RAPN), using data from randomised controlled trials (RCTs) or covariate-matched studies (propensity score-matched or matched-pair analysis).

Methods: A Preferred Reporting Items for Systematic Reviews and Meta-Analyses-compliant literature review was conducted on PubMed, EMBASE, Scopus and CENTRAL for relevant studies comparing off-clamp to on-clamp RAPN. Primary outcomes were estimated blood loss, postoperative percentage decrease in estimated glomerular filtration rate (eGFR), and margin positive rate.