The pheocromocytoma that occurs in Multiple endocrine neoplasia type 2A (MEN-2A), also known as Sipple's syndrome, is often bilateral and multicentric. The use of Meta-iodobenzylguanidine (M-IBG) scintigraphy is a safe and efficacious techinique for the location of suspected pheocromocytoma, especially for the cases of nonadrenal sites, recurrence postoperative and malignant metastatic disease. We present the case of a 26-years-old female patient with MEN-2A and bilateral adrenal pheocromocytoma (visualized by Magnetic Nuclear Ressonance and confirmed by histopatological exam), whose MIBG scintigraphy showed marked differentiated uptake of the adrenals (minimal on the right and more intense on the left). Relevant is the fact that the right tumor is much larger (5.5 cm of diameter) than the left (3.0 cm), functionally equal and with similar histology. We concluded that in the cases of MEN-2A, a minimal or negative M-IBG uptake does not exclude the need of bilateral surgical exploration of the adrenals.
Pheocromocytoma; Multiple endocrine neoplasia type 2A; Meta-iodobenzylguanidine; Sipple's syndrome
