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Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Localized Pheochromocytoma Treatment

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Evidence

In a single-institution study involving 56 patients with pheochromocytoma, 57% of patients (i.e., 17 of 30 patients) who underwent one or more cortical-sparing adrenalectomies avoided the need for routine steroid replacement; the clinical recurrence rate was low (i.e., 3 of 30 patients) and none of the patients developed metastatic disease.[5][Level of evidence: 3iiDii]

A similar approach may be reasonable in other hereditary pheochromocytoma-paraganglioma syndromes that are characterized by benign disease, but there are currently insufficient data upon which to base unequivocal recommendations. (Refer to the PDQ summary on the Genetics of Medullary Thyroid Cancer for more information on the treatment of inherited pheochromocytoma.)

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with localized benign pheochromocytoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References:

  1. Walz MK, Alesina PF, Wenger FA, et al.: Posterior retroperitoneoscopic adrenalectomy--results of 560 procedures in 520 patients. Surgery 140 (6): 943-8; discussion 948-50, 2006.
  2. Gagner M, Breton G, Pharand D, et al.: Is laparoscopic adrenalectomy indicated for pheochromocytomas? Surgery 120 (6): 1076-9; discussion 1079-80, 1996.
  3. Lee JE, Curley SA, Gagel RF, et al.: Cortical-sparing adrenalectomy for patients with bilateral pheochromocytoma. Surgery 120 (6): 1064-70; discussion 1070-1, 1996.
  4. de Graaf JS, Dullaart RP, Zwierstra RP: Complications after bilateral adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia type 2--a plea to conserve adrenal function. Eur J Surg 165 (9): 843-6, 1999.
  5. Yip L, Lee JE, Shapiro SE, et al.: Surgical management of hereditary pheochromocytoma. J Am Coll Surg 198 (4): 525-34; discussion 534-5, 2004.

This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http:// cancer .gov or call 1-800-4-CANCER.

WebMD Public Information from the National Cancer Institute

Last Updated: September 04, 2014
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
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