Molecular and Cellular Endocrinology, Journal Year: 2024, Volume and Issue: 592, P. 112344 - 112344
Published: Aug. 31, 2024
Language: Английский
British journal of surgery, Journal Year: 2024, Volume and Issue: 111(1)
Published: Jan. 1, 2024
Adrenocortical cancerACC is an exceedingly rare tumour, with estimated incidence of 1-2 per million population year, and overall 5-year survival rate only 30 cent.The ranges from 60 to 80 cent for tumours confined the adrenal gland, 35 50 locally advanced disease, much lower patients metastatic disease (below cent) 3 .
Language: Английский
Citations
10
Endocrine Reviews, Journal Year: 2024, Volume and Issue: 45(4), P. 521 - 552
Published: Feb. 20, 2024
Abstract Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors derived from neural crest cells adrenal medullary chromaffin tissues extra-adrenal paraganglia, respectively. Although the current treatment for PPGLs is surgery, optimal options advanced metastatic cases have been limited. Hence, understanding role of immune system in PPGL tumorigenesis can provide essential knowledge development better therapeutic tumor management strategies, especially those with PPGLs. The first part this review outlines fundamental principles microenvironment, their cancer immunoediting, particularly emphasizing We focus on how unique pathophysiology PPGLs, such as high molecular, biochemical, imaging heterogeneity production several oncometabolites, creates a tumor-specific microenvironment immunologically “cold” tumors. Thereafter, we discuss recently published studies related to reclustering based signature. second discusses future perspectives management, including immunodiagnostic promising immunotherapeutic approaches converting into active or “hot” known immunotherapy response patient outcomes. Special emphasis placed potent immune-related strategies signatures that could be used reclassification, prognostication, these improve care prognosis. Furthermore, introduce currently available immunotherapies possible combinations other therapies an emerging targets hostile environments.
Language: Английский
Citations
10
Journal of Neuroendocrinology, Journal Year: 2025, Volume and Issue: unknown
Published: March 5, 2025
Pheochromocytoma and paraganglioma (PPGL) are rare life-threatening tumors of the adrenal medulla extra-adrenal paraganglia, respectively. Management PPGL depends on accurate diagnosis treatment. This review provides a very comprehensive overview nuclear medicine in with some our perspective views diagnostic challenges pitfalls, treatment, new phase studies. It describes techniques including 64Cu/68Ga-DOTA-SSA, 18F-FDOPA, 18F-FDG, 123I-MIBG, emerging molecular imaging agents PRRT therapies, such as 203Pb VMT-α-NET 212Pb targeted alpha therapy (TAT). also insight into use proliferating cell antigen (PCNA) inhibitors combination therapeutics aggressive/metastatic PPGL. Through latest developments clinical practice, this aims to guide healthcare professionals improving accuracy therapeutic efficacy
Language: Английский
Citations
1
The Journal of Clinical Endocrinology & Metabolism, Journal Year: 2024, Volume and Issue: 109(9), P. 2366 - 2388
Published: April 23, 2024
Paragangliomas can metastasize, posing potential challenges both in symptomatic management and disease control. Systemic targeted radiotherapies using 131I-MIBG 177Lu-DOTATATE are a mainstay the treatment of metastatic paragangliomas. This clinical scenario discussion aim to enhance physicians' knowledge stepwise approach treat these patients with paraganglioma-targeted radiotherapies. It comprehensively discusses current approaches selecting paraganglioma for how choose between two based on specific patient tumor characteristics, when either therapy is feasible, or one superior another. The safety, efficacy, toxicity profiles, optimization also discussed, along other therapeutic options including radiotherapies, available besides therapies. Perspectives outlined since they hold promising near future that improve outcomes.
Language: Английский
Citations
6
The Journal of Clinical Endocrinology & Metabolism, Journal Year: 2025, Volume and Issue: unknown
Published: Jan. 2, 2025
Abstract Context Pheochromocytomas and paragangliomas (PPGLs) are rare endocrine tumors that frequently produce catecholamines. Catecholamine-induced cardiometabolic complications substantially contribute to increased morbidity mortality in PPGL patients prior surgical resection. Objective To determine whether markers of elevated risk persist following Design Retrospective analysis a multicenter cohort with PPGLs participating the prospective ProsPheo study ENS@T registry. Methods Cardiometabolic factors, including glycemic status, dyslipidemia, BMI, were assessed at diagnosis during follow-up. Patients history resected compared control group non-functioning adrenal adenomas (NFAA) from Results present or (n=188), metastatic (n=27) known susceptibility gene pathogenic variant (PV) for development without (n=44), included. We asymptomatic PV carriers PPGL: those showed significantly higher prevalence hyperglycemic disorders (p=0.013) carriers. In least 12 months follow-up post-surgery (n=113), (p<0.001), as well mean HbA1c (5.63%, SD 0.43%), higher, NFAA (n=76) similar age BMI (HbA1c 5.45%, 0.40%; p = 0.004). Conclusions Glycemic disturbances long-term after resection PPGL.
Language: Английский
Citations
0
Cancer Letters, Journal Year: 2025, Volume and Issue: unknown, P. 217464 - 217464
Published: Jan. 1, 2025
Language: Английский
Citations
0
Journal of Experimental & Clinical Cancer Research, Journal Year: 2025, Volume and Issue: 44(1)
Published: March 5, 2025
Neuroendocrine neoplasms (NENs) comprise a group of rare tumors originating from neuroendocrine cells, which are present in both endocrine glands and scattered throughout the body. Due to their scarcity absence specific markers, diagnosing NENs remains complex challenge. Therefore, new biomarkers required, ideally, easy-to-obtain blood samples. A panel soluble immune checkpoints (sPD-L1, sPD-L2, sPD-1, sCD25, sTIM3, sLAG3, Galectin-9, sCD27, sB7.2 sSIGLEC5) cytokines (IL4, IL6, IP10 MCP1) was quantified cohort 139 NENs, including 29 pituitary 46 pheochromocytomas paragangliomas, 67 gastroenteropancreatic pulmonary (GEPP) as well 64 healthy volunteers (HVs). The potential these circulating immunological parameters distinguish HVs, differentiate among various subtypes, predict prognosis evaluated using mathematical regression models. These factors-based models generated scores that were by Receiver Operating Characteristic (ROC) Area Under Curve (AUC) analyses. Correlations between clinical data performed. From analyses, minimal signature emerged, comprising five shared factors across models: Galectin-9. This refined evaluated, validated, checked for specificity against non-neuroendocrine tumors, demonstrating its clinically relevant tool identifying distinct NENs. Most analyzed showed expression patterns different Scores based on signatures identified with high efficiency, showing AUCs ranging 0.948 0.993 depending comparison, accuracies 92.52% 95.74%. illustrated biological features similarity divergence gastrointestinal correlated features. Furthermore, demonstrated strong performance distinguishing metastatic exitus GEPP achieving sensitivities specificities 80.95% 88.89%. Additionally, an easy-to-implement successfully all AUC values exceeding 0.900, 84.11% 93.12%, internally validated discovery validation randomization strategy. findings highlight effectiveness accurately differentiating analysis presents promising liquid biopsy approach delivering critical insights diagnosis. study serves proof-of-concept innovative holds transform management malignancies, providing non-invasive effective method early detection disease monitoring.
Language: Английский
Citations
0
Journal of Clinical Medicine, Journal Year: 2025, Volume and Issue: 14(6), P. 1907 - 1907
Published: March 12, 2025
Background: Pheochromocytoma and paraganglioma are catecholamine-secreting tumors, rarely presenting with pheochromocytoma multisystem crisis (PMC), a life-threatening endocrine emergency. The severity of the condition includes refractory cardiogenic shock may therefore require use temporary mechanical circulatory support. aim this review is to describe incidence crises associated shock, physiopathological impact on myocardial function, role support (tMCS) in its management, outcomes specific population. Methods: For purpose narrative review, literature search PubMed was conducted as 16 November 2024. Medical Subject Headings (MeSH) terms used included extracorporeal circulation”, “Impella”, “pheochromocytoma”, “paraganglioma”, “cardiogenic shock”, combined Boolean “OR” “AND”. Data from case series, retrospective studies, systematic reviews were considered. Seven studies reporting 45 patients who developed PMC requiring tMCS included. Patients young, median age 43 years (range 25–65) at presentation. Most cases presented severe hemodynamic instability, blood pressure lability, rapid progression left ventricular dysfunction. Veno-arterial membrane oxygenation (VA-ECMO) most common stabilize patients, initiate treatments, and, some cases, provide during emergent surgery. duration VA-ECMO 4 days 1–7) reported mean in-hospital survival rate 93.5%. Following weaning, survivors showed full recovery ejection fraction (LVEF). Conclusions: cardiac dysfunction observed PMC-associated be but appears reversible. should considered eligible bridge recovery, treatment, or rates impressively high, suggesting possibly substantial risk publication bias.
Language: Английский
Citations
0
Annals of Medicine, Journal Year: 2025, Volume and Issue: 57(1)
Published: March 13, 2025
Introduction Since the Fourth edition of WHO classification, PPGLs have been recognized for their metastatic potential, though no clear features can accurately predict this behavior. The prognostic value Ki-67 in assessing risk progression, relapse, or metastasis remains debated.
Language: Английский
Citations
0
Nature Communications, Journal Year: 2025, Volume and Issue: 16(1)
Published: March 17, 2025
Abstract Hereditary SDHB -mutant pheochromocytomas (PC) and paragangliomas (PG) are rare tumours with a high propensity to metastasize although their clinical behaviour is unpredictable. To characterize the genomic landscape of these identify metastasis biomarkers, we perform multi-omic analysis on 94 from 79 patients using seven molecular methods. Sympathetic (chromaffin cell) parasympathetic (non-chromaffin PCPG have distinct profiles reflecting cell-of-origin biochemical profile. TERT ATRX -alterations associated metastatic an increased mutation load, transcriptional telomeric features. Most quiet genomes some co-operative driver events, including EPAS1 /HIF-2α mutations. Two mechanisms acquired resistance DNA alkylating chemotherapies identifiable; MGMT overexpression mismatch repair-deficiency causing hypermutation. Our comprehensive therefore identifies features disease treatment response, expanding our understanding neuroendocrine tumours.
Language: Английский
Citations
0