[Kidney full of stones, and an adrenal gland not quite normal]

Abstract

A 31-year-old patient was followed for cystinuria, justifying CT scans. In 2006, a tissue mass of 3cm of the right adrenal gland, homogeneous, measured at 3.5cm in 2007 was noted. Blood pressure was 90/61mmHg, without orthostatic hypotension. During the clinical interview, no discomfort, sweat attack, headache, or palpitation was reported by the patient. Hormonal assays did not favor a primary hyperaldosteronism, or a hypercortisolism. Adrenal androgens were normal. 24h normetanephrines urinary excretion and metanephrines to creatinine ratio were elevated. One year later, the patient remained asymptomatic and normotensive. Plasma levels of normetanephrine and metanephrine were increased and highly pathological. The tumor was 5cm long and fixed individually on MIBG scintigraphy and PET scan. Right adrenalectomy was performed. A pheochromocytoma was confirmed histologically. The peculiarity here was the absence of symptoms, especially arterial hypertension. This currently affects up to 47% of cases, particularly in the context of incidentaloma investigations and family screening. Indeed, 35 to 40% of pheochromocytomas are associated with a germline mutation, justifying a genetic analysis in this patient. Genetic analysis did not show any mutation associated with pheochromocytoma. At a 6-year interval pheochromocytoma did not reappeared while BP increased reaching hypertension threshold. The only documented recurrence was lithiasis.

Keywords: Adrenal gland; Arterial hypertension; Hypertension artérielle; Incidentaloma; Incidentalome; Metanephrine; Métanéphrine; Normétanéphrine; Pheochromocytoma; Phéochromocytome; Surrénale.