Life-threatening events in patients with pheochromocytoma

Abstract

Objective: Pheochromocytomas are rare chromaffin cell-derived tumors causing paroxysmal episodes of headache, palpitation, sweating and hypertension. Life-threatening complications have been described in case reports and small series. Systematic analyses are not available. We took an opportunity of a large series to make a survey. Design and methods: We analyzed records of patients diagnosed with pheochromocytomas in three geographically spread German referral centers between 2003 and 2012 (nZ135). Results: Eleven percent of the patients (ten women, five men) required in-hospital treatment on intensive care units (ICUs) due to complications caused by unsuspected pheochromocytomas. The main reasons for ICU admission were acute catecholamine induced Tako-Tsubo cardiomyopathy (nZ4), myocardial infarction (nZ2), acute pulmonary edema (nZ2), cerebrovascular stroke (nZ2), ischemic ileus (nZ1), acute renal failure (nZ2), and multi organ failure (nZ1). One patient required extracorporeal membrane oxygenation due to a hypertensive crisis with lung edema occurring during delivery (nZ1). Two patients died of refractory shock and pheochromocytomas were found postmortem. Two patients were treated by emergency surgery. Compared to pheochromocytoma patients without life-threatening events (nZ120), patients with complications had a significant larger maximal tumor diameter (7.0 vs 4.5 cm, P!0.01), higher levels of catecholamines (20-vs ninefold upper limit of normal, P!0.01), and tended to be younger (42 vs 51 years, PZ0.05). Conclusion: Although pheochromocytomas are rare, they are likely to be associated with a life-threatening situation. Clinicians have to be aware of these situations and perform a timely diagnosis.

Extracorporeal membrane oxygenation for pheochromocytoma-induced cardiogenic shock

“…This is, to our knowledge, the largest case series of pheochromocytoma-induced refractory cardiogenic shock. As previously reported [1, 2], the major clinical feature was the sequence of initial hypertensive crisis followed by hypotensive shock, probably caused by severe LV failure. Following ECMO implantation, all our patients suffered paroxysmal hypertension with wide and rapid blood pressure fluctuations.…”

“…Many cardiac manifestations of pheochromocytoma have been reported, including Takotsubo-like cardiomyopathy, myocardial infarction, pulmonary edema and/or hypertensive crisis [1], but only rarely inaugural refractory cardiogenic shock requiring VA-ECMO (Table 2). This is, to our knowledge, the largest case series of pheochromocytoma-induced refractory cardiogenic shock.…”

18F-FDOPA PET/CT Uptake Parameters Correlate with Catecholamine Secretion in Human Pheochromocytomas

“…In conclusion, PHEO are life-threatening tumors which are the source of significant morbidity in clinical practice [31]. Rather than assessing the total amount of catecholamines, it appears critical to improve our understanding of the mechanisms involved in the pattern of secretion displayed by these tumors, as PHEO-induced mortality mainly occurs during an adrenergic storm, when the tumors suddenly release considerable amounts of catecholamines [32].…”

Pheochromocytomas are diagnosed incidentally and at older age in neurofibromatosis type 1

“…PHEO may manifest with adrenergic symptoms and/or hypertension, some patients presenting with the classical triad of headaches, tachycardia and perspiration, or be discovered as an adrenal incidentaloma . Undiagnosed PHEO may lead to significant morbidity and mortality in cases of sudden catecholamine release that may be induced by general anaesthesia, drugs, labour, biopsy or manipulation of the tumour during surgery …”