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World Journal of Surgery

, Volume 32, Issue 5, pp 683–687 | Cite as

Sporadic Paraganglioma

  • James A. Lee
  • Quan-Yang DuhEmail author
Article

Abstract

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.

Keywords

Paraganglioma Adrenal Incidentaloma Catecholamine Excess Urine Metanephrines SDHD Gene 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Société Internationale de Chirurgie 2008

Authors and Affiliations

  1. 1.Department of SurgeryColumbia University Medical CenterNew YorkUSA
  2. 2.Surgical ServiceVA Medical CenterSan FranciscoUSA

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