Pheochromocytoma in multiple endocrine neoplasia type 2: European study

Abstract

Mean age at diagnosis of pheo was 39.5 years (range 14-68 years) in MEN 2A and 32.4 years (range 15-41 years) in MEN 2B patients. Pheo occurred first in 25.1% of the cases (2-15 years before diagnosis of MTC) and after MTC in 40.2% (2-11 years). In other cases (34.7%), MTC and pheo were diagnosed at the same time. Involvement was bilateral in 67.8% of cases. Malignancy was only 4%. Thirty-nine deaths occurred in these 300 patients, 64.1% were linked in pheo, 23.1% to MTC and 12.8% to other causes. Surgery was unilateral in 39.7% of the cases and bilateral adrenalectomy was the first procedure in 48.4%. A bilateral adrenalectomy in two steps had to be done in 11.9% of cases. In conclusion, these results justify systematic and prolonged biochemical screening of pheo during follow-up of MTC and address some questions about the best mode of surgery.

Pheochromocytoma: Current Approaches and Future Directions

“…The mean age of presentation of pheochromocytoma is 39.5 years (range, 14 -68) for MEN-2A and 32.4 years (range, 15-41) for MEN-2B [49]. Extra-adrenal disease is rarely seen with MEN-2 [49]. Adrenal pheochromocytoma can present as either unilateral or bilateral disease.…”

“…In 9%-27% of MEN-2 patients, pheochromocytoma is the first manifestation of the syndrome; of these patients, 35%-73% will have MTC diagnosed concurrently [48 -50]. The mean age of presentation of pheochromocytoma is 39.5 years (range, 14 -68) for MEN-2A and 32.4 years (range, 15-41) for MEN-2B [49]. Extra-adrenal disease is rarely seen with MEN-2 [49].…”

Genetics and clinical characteristics of hereditary pheochromocytomas and paragangliomas

“…In these studies, w63% of the patients displayed bilateral PCC and only 3% had malignant disease. The mean age at PCC presentation was 36 years, and PCC was diagnosed before MTC in 12-25% of the cases (Modigliani et al 1995. PGLs are very rare in MEN2 and were not reported in any of the above patients, but a few cases of sympathetic and parasympathetic PGL have been described (Neumann et al 1993, Nilsson et al 1999 Frequencies of different characteristics are given in relation to the total number of patients with tumors.…”

“…Four large studies of PCCs in MEN2 , Modigliani et al 1995, Quayle et al 2007, including a total of 514 MEN2 patients with PCCs (479 MEN2A and 35 MEN2B), are summarized in this review. In these studies, w63% of the patients displayed bilateral PCC and only 3% had malignant disease.…”

Cardiac involvement in pheochromocytoma

“…Pheochromocytoma can occur alone or as a component of multiple endocrine neoplasia type 2 (MEN 2) syndrome clinical expression of which is sometimes poor. 1 However, pheochromocytoma can be responsible for cardiovascular morbidity and mortality.…”