Sporadic paraganglioma.
Paraganglioma are rare chromaffin cell tumors that develop from the neural crest cells. Parasympathetic ganglia-derived tumors are found almost exclusively in the neck and skull base and typically do not secrete catecholamines. In contrast, most sympathetic ganglia-derived tumors are found in the abdomen and produce an excess of catecholamines. Those grow within the adrenal medulla and are known as pheochromocytoma. Other sympathetic paragangliomas are also known as extra-adrenal pheochromocytomas. They arise outside of the adrenal gland and can be found anywhere along the sympathetic chain from the base of the skull and neck (5% of cases) to the bladder and prostate gland (10%). We use the term paraganglioma to refer to extra-adrenal tumors and pheochromocytoma to refer to intra-adrenal tumors. The majority of sympathetic paragangliomas are familial, commonly associated with the SDHB and SDHD gene mutations. Many are malignant. Medical management of catecholamine-secreting paraganglioma is the same as for pheochromocytoma. Favorably located abdominal paragangliomas can be safely resected laparoscopically, but the operation is technically more challenging than that for adrenalectomy.
Lee
James A
JA
Department of Surgery, Columbia University Medical Center, 161 Fort Washington Avenue, New York, New York 10032, USA.
Duh
Quan-Yang
QY
D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D006258:Head and Neck Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D006801:Humans; D010235:Paraganglioma / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*