Endocrine Abstracts

Introduction and Objective: Pheochromocytomas are usually monocomponent tumors, but very rarely there are also composite pheochromocytomas, with another medullary component (benign or malignant) derived from the neural crest present. Even more rarely there are mixed corticomedullary tumors, with pheochromocytoma plus a cortical adrenal tumor, eventually causing primary aldosteronism or hypercortisolim. Our objective in this communication is to illustrate an infrequent variety of pheochromocytoma, its clinical implications and prognosis, based on a clinical case.

Design & Method: Review of the patient’s clinical record and the relevant literature.

Results: A 63-year-old male, previously diagnosed of obesity, type 2 diabetes mellitus, dyslipidemia and essential hypertension was admitted in Cardiology for ACS without ST-segment elevation; angiography revealed no coronary lesions. Four years later the patient was admitted in Internal Medicine for unexplained weight loss (about 20 kg). Blood pressure was well controlled with enalapril, amlodipine, hydrochlorothiazide and bisoprolol; LDL-cholesterol and HbA1C were also adequately controlled. Abdominal CT showed a solid, hyperdense, heterogeneous, indeterminate 4 cm mass in the right adrenal and a 1.7 cm hypodense mass labeled as adenoma in the left one. Biochemical testing for hypercortisolism and primary aldosteronism was negative. Plasma metanephrine/normetanephrine were 1045/546 pg/mL, and a MIBG scan/SPECT-CT showed a single abnormal uptake labeled as pheochromocytoma. After standard preoperative preparation, a robotic right adrenalectomy was performed: recovery was uneventful, and the patient is presently normotensive and asymptomatic with normal plasmatic metanephrines and catecholamines. The final pathology report described a single 40 × 34 × 30 mm mass, labeled as mixed corticomedullay tumor, with 75% pheochromocytoma and 25% cortical adrenal components, without extracapsular extension. There were no signs of malignancy in the cortical component, and the pheochromocytoma component scored 2/20 in the PASS risk scale and 2/10 in the GAPP risk scale (low risk for metastasis in both). Genetic testing is pending. Unfortunately there are no specific risk score scales available for adrenal mixed corticomedullary tumors, but the cortical component should not add to the low risk of malignancy of the medullary component.

Conclusion: Adrenal mixed corticomedullary tumors are extremely rare, owing to the presence of two distinct components of different embryonic lineage; less than 30 cases have been described. Besides hypertension the patients may present hypercortisolism, primary hyperaldosteronism or nonspecific symptoms like weight loss. Awareness of this entity is needed to avoid perioperative hypertensive crises or missing diagnosis such as Cushing’s syndrome or primary hyperaldosteronism in addition to pheochromocytoma.