Journal of Endocrinology and Metabolism, ISSN 1923-2861 print, 1923-287X online, Open Access
Article copyright, the authors; Journal compilation copyright, J Endocrinol Metab and Elmer Press Inc
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Case Report

Volume 2, Number 4-5, October 2012, pages 198-204

A Rare Case of De novo C634G Germline Mutation of the RET Proto-Oncogene in a Patient With MEN 2A Syndrome With Aggressive Pheochromocytoma


Figure 1.
Figure 1. Light microscopy in pheochromocytomas of right adrenal gland (hematoxylin and eosin stains). A: A large tumor nest surrounded by tumor in the “zellballen” growth pattern (magnification, × 4). B: Diffuse growth area showing spindle-shaped tumor cells (magnification, × 20). C: Mitotic figures (black arrows) are observed in the spindly growth area (magnification, × 40). D: Tumor cells with profound nuclear pleomorphism (magnification, × 40).
Figure 2.
Figure 2. Ki-67 immunohistochemical study of pheochromocytoma (right adrenal gland). Ki-67 (MIB-1) proliferation index shows increased proliferative activity (> 5%) (magnification, × 40).


Table 1. Dynamic Levels of 24-Hours Urinary Excretion of Catecholamines and Their Metabolites
Test (units)Reference rangePreoperative (- 6 weeks)Postoperative (+ 3 weeks)Postoperative (+ 25 weeks)
VMA: Vanillylmandelic acid.
VMA (mg/24 h)< 634.79.74.1
Norepinephrine (mg/24 h)15 - 10059910336
Epinephrine (mg/24 h)2 - 24546< 114.5
Dopamine (mg/24 h)52 - 480192481203
Normetanephrine (mg/24 h)88 - 64913,511760297
Metanephrine (mg/24 h)58 - 2038,007368162


Table 2. Postoperative Levels of Plasma Fractionated Free Metanephrines
Test (units)Reference rangePostoperative (+ 3 weeks)Postoperative (+ 25 weeks)
Metanephrines (nmol/L)<
Normetanephrines (nmol/L)< 0.90.650.41