Long-Term Octreotide Treatment is an Alternative to Adrenalectomy in Patients With Bilateral Pheochromocytoma in the Multiple Endocrine Neoplasia Type 2A Syndrome

Abstract

Abstract: Pheochromocytoma, a rare tumor arising from catecholamine-producing chromaffin cells in the adrenal medulla, can appear sporadically or be a part of a tumor syndrome, including multiple endocrine neoplasia type 2 (MEN 2). MEN 2 is a rare disorder characterized by the presence of medullary thyroid carcinoma (MTC) with pheochromocytoma and/or primary hyperparathyroidism. Clinical presentation requires biochemical confirmation of catecholamine oversecretion, followed by anatomic localization methods (computed tomography, magnetic resonance) and functional diagnostic procedures (metaiodobenzylguanidine scintigraphy, In–111-labeled octreotide), allowing the possibilities for radiotherapy in some cases. Laparoscopic adrenalectomy is the “standard” treatment for benign pheochromocytoma, including the bilateral forms. We present a case of a 35-year-old woman patient with a history of MTC and giant bilateral pheochromocytomas (left adrenal tumor: 11 × 9 cm, right adrenal tumor: 4 × 3 cm in size on magnetic resonance, respectively). She had multiple endocrine neoplasia type 2A confirmed by genetic testing. Because of religious reasons, patient refused surgical treatment. In–111-labeled scintigraphy was performed, which suggested the use of subcutaneous octreotide as an alternative to surgical therapy. We report 24 months of successful treatment with octreotide, which improved symptoms, decreased hormone secretion, and slightly induced a reduction in size of the tumor. Our case shows that an octreotide treatment of pheochromocytoma can be an acceptable alternative to surgery when operation is not possible.