Paragangliomas

Paragangliomas are rare, neuroendocrine tumors that arise from paraganglionic tissue. The paraganglia are clusters of specialized cells located along the carotid body, jugular bulb, adrenal gland, sympathetic ganglia, and other sites in the head, neck, and trunk. These small structures serve as oxygen and chemoreceptors, sensing changes in blood flow, pH, and pO2/pCO2 levels, and regulating a variety of autonomic functions such as blood pressure, heart rate, respiration, and digestion. When paraganglial cells become neoplastic, they can cause various symptoms depending on their location, size, and hormone production. Most paragangliomas are benign, but some can be malignant or metastatic. The diagnosis of paraganglioma requires a combination of clinical, radiological, and biochemical investigations, such as MRI, CT, MIBG, PET, catecholamine levels, genetic testing, and pathology. The treatment of paraganglioma depends on the tumor's characteristics and the patient's condition, as well as the expertise of the multidisciplinary team involved. The options include surgery, radiation therapy, embolization, chemotherapy, targeted therapy, and watchful waiting. The management of paragangliomas requires long-term follow-up care, including regular monitoring of blood pressure, hormone levels, and imaging studies. Neurological research into paragangliomas aims to elucidate the molecular and genetic mechanisms underlying the development, progression, and response to therapy of these tumors. Recent advances in genomics, epigenomics, transcriptomics, proteomics, and metabolomics have revealed new insights into the biology of paragangliomas and offered potential targets for tailored treatments. Novel therapies such as PARP inhibitors, angiogenesis inhibitors, mTOR inhibitors, and immune checkpoint inhibitors are being evaluated in clinical trials for advanced paragangliomas.

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