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Pheochromocytoma and Paraganglioma Treatment (PDQ®)

Health Professional Version
Last Modified: 10/24/2013

Recurrent Pheochromocytoma Treatment

Surgery
Palliative Therapy
Inherited Pheochromocytoma or Paraganglioma
Current Clinical Trials

Standard treatment options for recurrent pheochromocytoma include the following:

  1. Surgery.
  2. Palliative therapy.

After resection of a localized pheochromocytoma presumed to represent a benign tumor and documented normal postoperative biochemical testing, disease recurrence occurs in 6.5% to 16.5% of patients, and 50% of patients with disease recurrence develop metastatic disease.[1-3] Insufficient data exist to determine recurrence rates after complete surgical resection of regional or metastatic disease.

Surgery

Treatment for recurrent disease involves appropriate medical management (i.e., alpha-adrenergic blockade) followed by complete surgical resection, when possible.

Palliative Therapy

Palliation of symptoms, including those related to catecholamine excess and local mass effect, is the primary focus of treatment for disease that is not resectable.

The following are options for patients with local-regional or metastatic disease who are not considered candidates for surgical resection:

  • Chemotherapy.
  • Targeted therapies.
  • High-dose 131I-metaiodobenzylguanidine radiation therapy.
  • Ablation therapies.
  • Radiation therapy.

(Refer to the Metastatic Pheochromocytoma Treatment section of this summary for more information.)

Inherited Pheochromocytoma or Paraganglioma

Patients with inherited pheochromocytoma or paraganglioma are at risk for the development of recurrent disease in the form of additional primary tumors. Follow-up evaluation and management of additional primary tumors in such patients is essential. (Refer to the Localized Pheochromocytoma Treatment section of this summary for more information.)

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent 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. Plouin PF, Chatellier G, Fofol I, et al.: Tumor recurrence and hypertension persistence after successful pheochromocytoma operation. Hypertension 29 (5): 1133-9, 1997.  [PUBMED Abstract]

  2. van Heerden JA, Roland CF, Carney JA, et al.: Long-term evaluation following resection of apparently benign pheochromocytoma(s)/paraganglioma(s). World J Surg 14 (3): 325-9, 1990 May-Jun.  [PUBMED Abstract]

  3. Amar L, Servais A, Gimenez-Roqueplo AP, et al.: Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma. J Clin Endocrinol Metab 90 (4): 2110-6, 2005.  [PUBMED Abstract]