Endocrine Abstracts

JOINT1440

Introduction: Pheochromocytoma, a relatively rare (<0.05%), catecholamine-secreting tumor, is almost always lethal unless recognized and appropriately treated. Clinical and biochemical manifestations are mainly caused by excess circulating catecholamines and hypertension. Sustained or paroxysmal hypertension associated with headaches, sweating, or palpitations, occurs in 95% of patients, but at least 5% are normotensive. All patients with manifestations should be investigated for pheochromocytoma. Plasma free metanephrines and fractionated urinary metanephrines are the most sensitive (≊100%) chemical tests for diagnosing sporadic and familial pheochromocytomas. For suspected metastatic disease use of 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/CT scanning is recommended. 2 Surgical resection is successful in 90% of cases. Pheochromocytomas <5 cm in diameter can be removed laparoscopically; larger tumors should be removed by open surgery. Drug treatment prior to and during surgery is mandatory.

Care Report: 54 y/o male patient was hospitalized in our clinic due to hypertensive crises. Upon investigation recurrent pheochromocytoma was suspected and was recommended to repeat laboratory tests, including urinary and plasma metanephrines and normetanephrines, which revealed significantly elevated values and patient was advised to perform 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/CT. Further MRI imaging revealed nonhomogenous, hypovascular adrenal tumor of about 5-6 cm with suspected left renal artery and vein invasion. Imaging tests demonstrated recurrent disease. Patient was referred to a high volume adrenal surgeon. Laparoscopic adrenalectomy and ipsilateral nephrectomy was performed in short time with patient’s perioperative management by a multidisciplinary team approach. During a follow up visit 1 week after surgery patient doesn’t complain of increased blood pressure or palpitations, has no need of antihypertensive medications. Patient is advised to perform genetic testing to exclude familial pheochromocytoma. Follow up laboratory tests of metanephrines and normetanephrines are planned 4 weeks after surgery.

Conclusion: Pheochromocytomas are rare, mostly benign catecholamine-producing tumors of chromaffin cells of the adrenal medulla or of a paraganglion. If remaining unrecognized or untreated, they can be a life-threatening condition. Therefore, the most important message of this case report is to stress the importance of patient education about the diagnosis and follow up visits with the specialist. Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH, Naruse M, Pacak K, Young WF Jr; Endocrine Society. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline