Azzoug S. , Amirou Al. , Rabehi L. , Chentli F.

Department of Endocrine and Metabolic diseases, Bab El Oued Hospital, Algiers, Algeria
Author    Correspondence author
International Journal of Clinical Case Reports, 2015, Vol. 5, No. 21   doi: 10.5376/ijccr.2015.05.0021
Received: 14 Apr., 2015    Accepted: 28 May, 2015    Published: 09 Jun., 2015

This is an open access article published under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Azzoug et al., 2015, Malignant Pheochromocytoma, International Journal of Clinical Case Reports, Vol.5, No.21 1-4 (doi: 10.5376/ijccr.2015.05.0021)

Pheochromocytoma is malignant in approximately 10%. Malignancy is evidenced by the presence of metastases outside chromaffin tissue sites. We report here the observations of four malignant pheochromocytoma (two male and two female) encountered in our practice during a period of twenty five years. They were aged between 6 and 33 years at first presentation. Two were sporadic and two were linked to hereditary syndromes, one multiple endocrine neoplasia type 2 (MEN2) and one neurofibromatosis type 1. The adrenal tumor measured between 24 and 115 mm, it was bilateral in the MEN2 syndrome and unilateral in the reminder cases. Metastases were present at first presentation in two cases, in the immediate post operative course in one case and many years after primary surgery in thepatient presenting MEN2 syndrome. Malignant pheochromocytoma is rare; the diagnosis of malignancy may be present at first diagnosis but sometimes it may occur many years after primary surgery therefore lifelong monitoring is mandatory.

Malignant pheochromocytoma; Chromaffine tissue; Hereditary syndromes; Lifelong monitoring