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.[1,2,3] Insufficient data exist to determine recurrence rates after complete surgical resection of regional or metastatic disease.
Important concepts that should be understood by those treating and caring for a child who has a brain or spinal cord tumor include the following:
The cause of most childhood brain tumors remains unknown.
Selection of an appropriate therapy can only occur if the correct diagnosis is made and the stage of the disease is accurately determined.
Children with primary brain or spinal cord tumors represent a major therapy challenge that, for optimal results, requires the coordinated efforts...
Treatment for recurrent disease involves appropriate medical management (i.e., alpha-adrenergic blockade) followed by complete surgical resection, when possible. 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. Chemotherapy, targeted therapies, high-dose 131 I-metaiodobenzylguanidine radiation therapy, ablation therapies, and radiation therapy are options for patients with local-regional or metastatic disease who are not considered candidates for surgical resection. (Refer to the Metastatic Pheochromocytoma section of this summary for more information.)
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 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 and paraganglioma. 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.
Plouin PF, Chatellier G, Fofol I, et al.: Tumor recurrence and hypertension persistence after successful pheochromocytoma operation. Hypertension 29 (5): 1133-9, 1997.
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.
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.