Endocrine Abstracts

Background: Pheochromocytomas are rare tumors, with prevalence less than 0.2% of hypertensive patients, in general population being unknown. With increasing access to imaging and hormonal workup, more pheochromocytomas are diagnosed. This may have changed the occurrence of the classic presentation with hypertension and the classic triad.

Methods: We present 3 cases of pheochromocytoma diagnosed in our departments over the last 2 years, with 3 different phenotypes: the classic form, a resistant form of secondary hypertension and a normotensive patient.

Case reports: Case 1- classic triad: A 62 years old man was referred with classic symptoms: headache, palpitations, diaphoresis, severe hypertension, with paroxysmal rises. Hormonal workup confirmed the diagnosis: elevated metanephrines and normetanephrines level, dyslipidemia, hyperglycemia. The imagistic evaluation revealed right adrenal tumor. The histopathological and immunohistochemical exam suggested a malignant pheochromocytoma. After suprarenalectomy, we obtain a proper control of the arterial tension, with no paroxysm, normal hormonal profile. Case 2- resistant secondary hypertension: The second case is a 30 years old man, that complained of resistant hypertension, without a specific pattern and no other signs. He had a significant family history of cardio-vascular diseases, both his parents died in their 30’s. Hormonal profile shown pheochromocytoma: elevated normetaphrines level with normal metanephrines, normal aldosteron/renin ratio, normal Dexamethasone suppression of Cortisol; normal calcemia and PTH level. Imagistic evaluation identified right adrenal tumor. The patient was treated with doxazosin and perindopril/indapamid/amlodipin association. Despite high normal calcitonin level, he refused all other investigation, therefore no data about a genetic disorder is available. Case 3- normotensive: A 57 years old woman, with history of thyroidectomy for benign nodular goiter, was evaluated for an inferior deep cervical mass; she presented mild tachycardia, anxiety and insomnia. Hormonal workup: normal metanephrines level and elevated plasma and urinary normetanephrines level, high cromogranine A and serotonine level; normal Calcitonin and PTH level, without any other sign of a genetic disorder. CT/MRI scans describes 3 masses: an inferior deep cervical tumor, a paravertebral thoracic lesion and left adrenal mass. After treatment with doxazosin well tolerated, the surgical cure with histopathological and immunohistochemical exam revealed a benign neck schwannoma and adrenal pheochromocytoma; the patient is still in investigation for genetic disorders, given association of schwannoma with pheochromocytoma.

Conclusion: Though hypertension is common, hypo to normotensive presentation is possible. The spectrum of the presentation of pheochromocytoma continues to expand. In some cases, genetic disorders should be considered.