Indication

Malignant paraganglioma

Malignant paraganglioma is a rare neuroendocrine neoplasm arising from extra-adrenal chromaffin or non-chromaffin neural crest tissue within the sympathetic or parasympathetic nervous systems. Read more about Malignant paraganglioma

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Common Malignant paraganglioma therapies.

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WELIREG (belzutifan)

ANTINEOPLASTIC - HYPOXIA INDUCIBLE FACTOR (HIF) INHIBITORS·RENAL CELL CARCINOMA·+3
Merck Sharp & Dohme
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About Malignant paraganglioma.

Malignant paraganglioma is a rare neuroendocrine neoplasm arising from extra-adrenal chromaffin or non-chromaffin neural crest tissue within the sympathetic or parasympathetic nervous systems.

Pathophysiologically, malignancy is defined by the presence of distant metastases in non-chromaffin tissues where paraganglionic cells are normally absent, most frequently affecting bones, regional lymph nodes, the liver, and lungs.

Clinically, these tumors can develop in adults or children and may be sporadic or linked to germline mutations, such as in the succinate dehydrogenase genes.

Sympathetic lesions typically produce excessive catecholamines leading to paroxysmal hypertension, headaches, diaphoresis, and tachycardia, whereas parasympathetic variants usually arise in the head and neck and predominantly present with painless local masses or cranial nerve deficits.

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