Long-term evaluation following resection of apparently benign pheochromocytoma(s)/paraganglioma(s)

In the absence of distant disease, the pathologic diagnosis of malignancy in pheochromocytoma or paraganglioma is impossible. In an effort to establish the true incidence of recurrence in this disease, we have analyzed long-term follow-up (average, 15.8 years) of 98 patients who underwent complete resection of localized, noninvasive, histologically-benign pheochromocytomas and paragangliomas at our institution between 1960 and 1976. Eighty-eight patients had nonfamilial, sporadic pheochromocytoma/paraganglioma. Nine had multiple endocrine neoplasia (MEN) type 2 (2A: 7, 2B: 2), and 1 had familial pheochromocytoma. Seventy-nine patients had single pheochromocytomas; 10 had single extraadrenal tumors (paragangliomas); and 9 had multicentric or bilateral adrenal tumors. Six patients (6.5%) developed recurrent pheochromocytoma after documentation of normal postoperative urinary catecholamine levels. One of these patients had MEN 2A. The recurrences developed at intervals from 5 to 13 years following initial resection. These were distant in 3 patients, local in 2, and both local and distant in a single patient. None of the recurrences occurred in the 13 patients who, on pathologic rereview, had either local or vascular invasion. No paraganglioma recurred. Life-long follow-up of all patients who have had pheochromocytomas or paragangliomas resected is mandatory.