Nuclear medicine and perspective thoughts in the diagnosis and treatment of pheochromocytoma and paraganglioma

Journal of Neuroendocrinology, Год журнала: 2025, Номер unknown

Опубликована: Март 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

Язык: Английский

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Neoadjuvant Intratumoral MBT(A) Immunotherapy Prevents Distant Metastases and Recurrence in Murine Models

Cancer Letters, Год журнала: 2025, Номер unknown, С. 217464 - 217464

Опубликована: Янв. 1, 2025

Язык: Английский

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Systemic Therapy for Patients with Metastatic Pheochromocytoma and Paraganglioma

Best Practice & Research Clinical Endocrinology & Metabolism, Год журнала: 2025, Номер unknown, С. 101977 - 101977

Опубликована: Янв. 1, 2025

Язык: Английский

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A blood-based liquid biopsy analyzing soluble immune checkpoints and cytokines identifies distinct neuroendocrine tumors

Journal of Experimental & Clinical Cancer Research, Год журнала: 2025, Номер 44(1)

Опубликована: Март 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.

Язык: Английский

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MAML3-fusions modulate vascular and immune tumour microenvironment and confer high metastatic risk in pheochromocytoma and paraganglioma

Best Practice & Research Clinical Endocrinology & Metabolism, Год журнала: 2024, Номер 38(6), С. 101931 - 101931

Опубликована: Авг. 29, 2024

Pheochromocytomas and paragangliomas are rare neuroendocrine tumours. Around 20-25 % of patients develop metastases, for which there is an urgent need prognostic markers therapeutic stratification strategies. The presence a MAML3-fusion associated with increased metastatic risk, but neither the processes underlying disease progression, nor targetable vulnerabilities have been addressed. We compiled cohort 850 patients, has shown 3.65 fusion prevalence represents largest MAML3-positive series reported to date. While MAML3-fusions mainly cause single pheochromocytomas, we also observed somatic post-zygotic events, resulting in multiple tumours same patient. MAML3-tumours show expression neuroendocrine-to-mesenchymal transition markers, MYC-targets, angiogenesis-related genes, leading distinct tumour microenvironment unique vascular immune profiles. Importantly, our findings identified specific beyond Wnt-pathway dysregulation, such as rich network, overexpression PD-L1 CD40, suggesting potential targets.

Язык: Английский

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Overview of recent guidelines and consensus statements on initial screening and management of phaeochromocytoma and paraganglioma in SDHx pathogenic variant carriers and patients

Best Practice & Research Clinical Endocrinology & Metabolism, Год журнала: 2024, Номер unknown, С. 101938 - 101938

Опубликована: Сен. 1, 2024

Язык: Английский

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Metastatic pheochromocytoma and paraganglioma: Integrating tumor biology in clinical practice

Molecular and Cellular Endocrinology, Год журнала: 2024, Номер 592, С. 112344 - 112344

Опубликована: Авг. 31, 2024

Язык: Английский

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Recent progress in molecular classification of phaeochromocytoma and paraganglioma

Best Practice & Research Clinical Endocrinology & Metabolism, Год журнала: 2024, Номер unknown, С. 101939 - 101939

Опубликована: Сен. 1, 2024

Язык: Английский

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Alterations and clinical value of peripheral lymphocyte subsets and inflammatory factors in paragangliomas

The Journal of Clinical Endocrinology & Metabolism, Год журнала: 2024, Номер unknown

Опубликована: Окт. 7, 2024

Abstract Objectives Given the pivotal role of immune and inflammatory responses in tumor patients, present study aimed to explore alterations clinical value peripheral lymphocyte subsets factors pheochromocytomas/paragangliomas (PPGLs). Methods The data 327 including 102 patients with metastatic PPGLs, were retrospectively analyzed. Peripheral determined by flow cytometry. Relationships between parameters clinicopathological characteristics evaluated intergroup comparisons correlation analyses. Univariate multivariate logistic regression analyses employed identify indicators. corresponding nomogram was constructed for discrimination calibration. Results median age at diagnosis 45.0 years, duration follow-up 3.0 years. Compared those younger (< years), most significantly reduced older (≥ 45 years) (P < .05). count lymphocytes, CD3+ T cells CD4+ negatively correlated 24-hour urinary epinephrine plasma metanephrine levels (R = −0.2 ∼ 0.1, P In addition, lymph node (n 37) or bone metastases 41) had a lower percentage Multivariate analysis revealed that cell ≥ 1446.50/μL, cell% 39.95%, CD8+ 24.95%, CD4+/CD8+ ratio 2.88, B 8.65%, TNF-alpha 12.45 pg/mL, IL-8 30.50 platelets 269.50 × 109/L significant indicators PPGLs. area under curve (AUC) 0.800 (95% CI: 0.736-0.865). Conclusion Immunosenescence, characterized dysfunction aging, observed Higher might impair host response. Monitoring changes serum cytokines could indicate patients’ conditions, especially occurrence metastasis.

Язык: Английский

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Role of B cells in intratumoral MBTA immunotherapy of murine pheochromocytoma model

Best Practice & Research Clinical Endocrinology & Metabolism, Год журнала: 2024, Номер unknown, С. 101941 - 101941

Опубликована: Сен. 1, 2024

Язык: Английский

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