Pheochromocytomas and Paragangliomas-Current Management.

Area of Science:

• Endocrinology
• Oncology
• Genetics

Background:

• Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors originating from the paraganglion system.
• PPGLs are typically slow-growing and non-malignant, with 90% occurring in the adrenal glands (pheochromocytomas).
• Approximately 25% of PPGLs are linked to germline mutations, increasing the risk of multifocal and metastatic disease.

Purpose of the Study:

• To review current knowledge on the etiopathogenesis of PPGLs.
• To summarize diagnostic approaches for PPGLs, including laboratory, biochemical, and imaging methods.
• To provide an overview of contemporary therapeutic strategies for PPGL patients.

Main Methods:

• Literature review of etiopathogenesis, diagnostics, and therapy for PPGLs.
• Focus on current management options for locoregional and metastatic PPGLs.
• Inclusion of information on surgical and non-surgical treatment modalities.

Main Results:

• PPGLs are classified into sympathetic and parasympathetic types, with varied anatomical distributions.
• Diagnostic tools include laboratory, biochemical, and advanced imaging techniques.
• Treatment strategies range from surgery for locoregional disease to systemic therapies for metastatic PPGLs.

Conclusions:

• PPGLs require a multidisciplinary approach for diagnosis and management.
• Current therapies offer good prognosis, with over 90% 5-year survival rate.
• This review emphasizes the latest advancements in PPGL management.