Stage Information for Pheochromocytoma and Paraganglioma
There is no standard staging system for pheochromocytoma and paraganglioma. Patients have traditionally been divided into categories based on the presence of localized (apparently benign), regional, and metastatic disease.
There are no clear data regarding the survival of patients with localized (apparently benign) disease or regional disease. Although patients with localized (apparently benign) disease should experience an overall survival approaching that of age-matched disease-free individuals, 6.5% to 16.5% of these patients will develop a recurrence, usually 5 to 15 years after initial surgery.[1,2,3]
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Approximately 50% of patients with recurrent disease experience distant metastasis. The 5-year survival in the setting of metastatic disease (whether identified at the time of initial diagnosis or identified postoperatively as recurrent disease) is 40% to 45%.
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.