Normotensive Pheochromocytoma: Institutional Experience

Abstract

We report an audit of nine cases of subclinical or normotensive pheochromocytoma managed in our department. This is the first report from India of such a series. During the period 1990-2003 a total of 45 patients of pheochromocytoma were diagnosed and managed in the Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences. Among them, nine patients were diagnosed as having subclinical or normotensive pheochromocytoma. Inclusion criteria for normotensive pheochromocytoma were: no previous history of hypertension clinically and, in the previous treatment documents, any episode of symptoms suggesting high blood pressure. The demographic profile, clinical presentation, biochemical investigations, imaging results, surgical notes, and follow-up record were reviewed retrospectively. All patients had a primary complaint of flank/abdominal pain and were normotensive. Seven had elevated urinary metanephrine levels, and one patient had normal values. One patient did not undergo the urinary metanephrine assay. Imaging of the abdomen showed seven adrenal and two extra-adrenal masses (eight had computed tomography scans of the abdomen, and one underwent ultrasonography). After selective alpha-adrenergic blockade (prazosin), surgery was performed. Six patients required infusion of sodium nitroprusside intraoperatively. The final histopathology was pheochromocytoma in all patients. Metanephrine levels were normal during the follow-up. Normotensive pheochromocytomas are a distinct entity, and all adrenal incidentalomas should be investigated for catecholamine hypersecretion. We support the use of preoperative alpha-adrenergic blockade, and these patients should be treated along the same lines as hypertensive pheochromocytoma.

Preoperative alpha blockade for normotensive pheochromocytoma

“…Normotensive pheochromocytoma refers to the situation in which a proven pheochromocytoma is totally asymptomatic. Approximately, 10% of histologically proven benign pheochromocytomas are incidentally discovered by radiologic examination in the absence of symptoms [3]. Pharmacologic preparation with drugs that block adrenoreceptors or decrease catecholamine synthesis is necessary for hemodynamic stability.…”

Intraoperative diagnosis of functional retroperitoneal multiple paraganglioma: A case report

“…It is considered that 30% of functional paraganglioma patients have normal blood pressure (7). The possible reasons for this have been explained by Agarwal et al (8). In our study, the patient had normal blood pressure and did not receive a metanephrine examination, with a diagnosis of retroperitoneal tumor.…”

Challenges for endocrine surgeons: management of a colossal normotensive pheochromocytoma

“…4 Shao Y et al in 2011 described preoperative α-blockade has no advantage in sustaining hemodynamic stability in patients with normotensive pheochromocytoma and can augment the use of vasopressor drugs and colloid infusion. 5 Howbeit we advocate α-blockade prior to surgery because it is imperative to recognize even in normotensive pheochromocytoma abysmal intraoperative hemodynamic fluctuation can occur and result in life threatening pheochromocytoma crisis.…”