Paraganglioma

A neuroendocrine tumor arising from paraganglia, cell groups associated with the autonomic nervous system. Some paragangliomas release catecholamines, while others do not.

Paraganglia: Clusters of neuroendocrine cells associated with the autonomic nervous system, distributed inside and outside the adrenal glands. These cell groups are the tissue of origin for paragangliomas.
Sympathetic paraganglioma: A paraganglioma arising along sympathetic paraganglia, often in the abdomen or thorax and sometimes secreting catecholamines. Its sympathetic origin helps explain hormone-related symptoms and common tumor locations.
Plasma free metanephrines: Blood measurements of metanephrine and normetanephrine used to detect catecholamine-producing tumors. They are a sensitive initial test when a secreting paraganglioma is suspected.
SDHB: A gene encoding a subunit of succinate dehydrogenase; pathogenic variants can predispose to paraganglioma and other tumors. SDHB variants are associated with extra-adrenal tumors and increased metastatic risk.
Paraganglioma symptoms: Clinical effects of paraganglioma, ranging from catecholamine-related episodes to symptoms caused by local tumor growth. The symptom pattern differs between secreting tumors and nonsecretory head and neck tumors.
Chromaffin cell: A neuroendocrine cell that stores and releases catecholamines, typically in the adrenal medulla or extra-adrenal paraganglia. Many paragangliomas resemble chromaffin cells and can secrete their hormones.
Head and neck paraganglioma: A paraganglioma arising from parasympathetic paraganglia in the head or neck, usually without catecholamine secretion. These tumors often present through local growth rather than hormone excess.
Normetanephrine: A metabolite formed from norepinephrine that is measured in blood or urine. Elevated levels can indicate norepinephrine secretion by a paraganglioma.
SDHD: A gene encoding a subunit of succinate dehydrogenase, with pathogenic variants linked especially to head and neck paragangliomas. Its parent-of-origin effects make family history particularly relevant.
Hypertensive crisis: A severe, potentially life-threatening elevation in blood pressure requiring urgent medical care. Unrecognized catecholamine release can trigger extreme blood pressure elevations.

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