Endocrine Abstracts

Introduction: Pheochromocytomas are rare catecholamine-producing neuroendocrine tumors that are usually benign, but which may also present as or develop into a malignancy. Predicting such behavior is notoriously difficult and there are currently no curative treatments for malignant tumors.

Case Report: A male patient aged 23 years old who was investigated for headaches, flushing and palpitations. The patient presented with classic clinical features of NF-1, café-au-lait spots and skin nodules. Laboratory tests revealed elevated catecholamine levels in the 24-h urine collection. Computed Tomography Scan revealed a mass measuring 10×8.2×9.4 cm in the left adrenal gland and pulmonary metastases. He was operated on and histological examination confirmed the diagnosis of pheochromocytoma with a Pass score of 9. After surgery, urinary catecholamines were high and there were diffuse pulmonary and liver asymptomatic metastases in Octreotide scan uptake. The I131 MIBG scan revealed the progression of pulmonary and liver metastases and revealed bone metastases. Radionuclide treatment with use of 131-I MIBG is indicated.

Discussion: Pheochromocytomas are tumors of the adrenal medulla and extra-adrenal chromaffin tissue that secrete catecholamines, resulting in hypertension, whether sustained or paroxysmal, and other symptoms of increased production of catecholamines. They may be classified as sporadic or familial. Most of the pheochromocytomas are sporadic. Familial predisposition is seen mainly in patients with multiple endocrine neoplasia type 2, neurofibromatosis Type 1 (NF-1), von Hippel-Lindau disease and familial carotid body tumors. Laparoscopic adrenalectomy (LA) has gained field in the surgery of the adrenals and nowadays it is the procedure of choice. A long-term follow-up is required to monitor for recurrence in patients with genetic predisposition. I-131 MIBG therapy has been widely used for metastatic or unresectable MIBG-avid pheochromocytomas and paragangliomas. Several groups have tried single or repeated therapy with a variable dose of I-131 MIBG.

Conclusion: The association of a malignant pheochromocytoma with neurofibromatosis type 1 although very rare should be known as pheochromocytoma and its metastases may be totally asymptomatic as in the presented case.

Keywords: Pheochromocytoma, metastasis-treatment