Endocrine Abstracts

Introduction

Pheochromocytomas originate in the adrenal medulla. Although rare, they can be lethal through their cardiovascular complications. They may be sporadic or come in the context of hereditary syndroms. The aim of our work is to describe the clinical, biological and radiological features of these tumors and assess the clinical outcomes after surgical treatment.

Materials and methods

Our study is retrospective descriptive, involving 21 patients presenting pheochromocytomas, followed in our Endocrinology-Diabetology and Nutrition Department.

Results

The mean age of our patients was 46.3 ± 16.3 years with a sex ratio H/F of 0.4. History of hypertension was present in 52.3% of cases and diabetes in 46% of cases. In 52.3% of patients the pheochromocytoma was discovered while exploring adrenal incidentaloma, 4.7% in the context of inherited disorders, especially multiple endocrine neoplasia type 2, and 42.8% in the course of symptoms. 52.6% of cases presented with the complete Menard Triad, 36.8% reported abdominal pain, and only 26% experienced weight loss. All of them had elevated 24-h urinary fractionated metanephrines, varying from 4 up to more than 100 times the normal value, with an average of respectively 12 and 11 times the normal value for metanephrine and normetanephrine. Abdominal CT scan was performed for all patients revealing unilateral tumors in 79% of cases and bilateral in 21% of cases. The mean size of the tumor was 42.1 ± 26.2 mm (11–103). Before surgery, 38% of our patient received α1blockers preparation 15 days before surgery. During the intervention, only 37% presented hypertensive peaks without further complications. In post-operative follow-up, a good blood pressure control was achieved and antihypertensive treatment was reduced in all of our hypertensive patients. As for glycaemic control, the mean HbA1c dropped from 8.1% ± 1.7 before surgery to 6.6% ± 1.3 three months after. Antidiabetic treatment was permanently stopped in 22% of cases concluding to the diagnosis of secondary diabetes mellitus, and significantly reduced in the rest. In our series, 71.4% of pheochromocytomas were sporadic, 23.8% secondary to MEN1 and only 4.7% in Van Hippel Lindau Disease.

Discussion–conclusion

Pheochromocytomas are rare but dangerous tumors due to the catecholalmines over production and the potential cardiovascular complications. The preoperative treatment with α1blockers at least 10–14 days before surgery prevents and limits peroperative hypertensive peaks, arrhythmia and circulatory complications. Surgery remains the gold standard treatment with good post-surgical outcomes. Pheochromocytomas may be associated with familial syndroms like NEM2 and VHL or hereditary mutations of succinate dehydrogenase subunits (SHD). Therefore, genetic testing seems relevant especially if the patient present a familial history of pheochromocytomas.