MEN (multiple endocrine neoplasia) type 2A syndrome, a rare autosomal dominant condition of RET proto-oncogene gain-of-function mutations mostly involves medullar thyroid cancer and pheocromocytoma (40-50%) while primary hyperparathyroidism has a less frequent incidence (based on genotype-phenotype correlations).We introduce a female case of MEN 2A syndrome with a particular evolution.This is a case report.A 38-old year female with Rectangular Cake Pans family history of MEN2A syndrome, is currently experiencing transitory flush associated with mild palpitations (maximum 135/80 mmHg, respective 100-100 beats/minute).
6 years ago she had total thyroidectomy for medullar thyroid cancer (normal plasma metanephrines/normetanephrines and adrenal aspects at computed tomography).2 years later she was detected with a unilateral pheocromocytoma.Laparoscopic left adrenalectomy was done.Currently, only an increase of plasma metanephrines is postive (twice normal upper limits), not plasma normetanephrines, nor urinary 24-hour metanephrines and normetanephrines.
Computed tomography showed a right adrenal tumor of 1.3/2.3 cm and a left adrenal tumor of 1.12/0.
76 cm.Whole body MIBG (meta-iodo-benzyl guanidine) scintigram was done and confirmed bilateral activity.Bilateral adrenalectomy is necessary.Flush after partial adrenalectomy in patient with prior thyroidectomy for MEN2A syndrome - associated medullar thyroid cancer underlines Pressure Switch Hose pheocromocytoma.
The newly detected bilateral adrenal masses require an adequate differential diagnosis of post-operatory aspect thus the usefulness of MIBG scintigram.