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A paraganglioma is a rare neuroendocrine neoplasm that may develop at various body sites (including the head, neck, thorax and abdomen). When the same type of tumor is found in the adrenal gland, they are referred to as a pheochromocytoma.
Carney triad (CT), named for J Aidan Carney, is considered to be a specific type of multiple endocrine neoplasia (MEN). The three classically associated tumors are a subset of gastric epithelioid leiomyosarcoma (it is now known that this subset is actually gastrointestinal stromal tumor arising from the interstitial cells of Cajal), pulmonary chondroma, and extra-adrenal paraganglioma.
Adrenal pheochromocytomas are usually benign while extra-adrenal ones are more malignant. [3] They are most of the time in the adrenals, and only rarely outside of the abdomen. They usually secrete hormones and estimates of a familial history vary. [1] [2] Nonchromaffin paragangliomas are usually benign. [3]
Patients with a paraganglioma are more likely to develop metastases than those with a pheochromocytoma. [134] The most common extra-adrenal sites of metastases are the lymph nodes, lung, liver, and bone. [135]
These terms can be used interchangeably but usually paraganglioma refer to a tumor originating from chromaffin cells outside the adrenal gland, which can also be called extra-adrenal pheochromocytoma, whereas pheochromocytoma typically refer to a tumor originating from the chromaffin cells within the adrenal gland. [2]
He says Schaetzel told him he believes Floyd died not from asphyxia from Chauvin’s actions, but from complications of a rare tumor called a paraganglioma that can cause a fatal surge of adrenaline.
It can be the source of paraganglioma. [8]The organ of Zuckerkandl is of pathological significance in the adult as a common extra-adrenal site of pheochromocytoma though the most common extra-adrenal site is in the superior para-aortic region between the diaphragm and lower renal poles.
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