Pheochromocytoma Crisis Is Not a Surgical Emergency

Division of Endocrinology and Metabolism (C.L., J.B.T.), Department of Medicine, University of California, San Francisco, California 94143.
The Journal of Clinical Endocrinology and Metabolism (Impact Factor: 6.21). 01/2013; 98(2). DOI: 10.1210/jc.2012-3020
Source: PubMed


Context:Pheochromocytoma crisis is a feared and potentially lethal complication of pheochromocytoma.Objective:We sought to determine the best treatment strategy for pheochromocytoma crisis patients and hypothesized that emergency resection is not indicated.Design:Retrospective cohort study (1993-2011); literature review (1944-2011).Setting:Tertiary referral center.Patients:There were 137 pheochromocytoma patients from our center and 97 pheochromocytoma crisis patients who underwent adrenalectomy from the literature.Intervention:Medical management of pheochromocytoma crisis; adrenalectomy.Main Outcome Measure(s):Perioperative complications, conversion, and mortality.Results:In our database, 25 patients (18%) presented with crisis. After medical stabilization and α-blockade, 15 patients were discharged and readmitted for elective surgery and 10 patients were operated on urgently during the same hospitalization. None underwent emergency surgery. Postoperatively, patients who underwent elective surgery had shorter hospital stays (1.7 vs 5.7 d, P = 0.001) and fewer postoperative complications (1 of 15 [7%] vs 5 of 10 [50%], P = 0.045) and were less often admitted to the intensive care unit (1 of 15 [7%] vs 5 of 10 [50%], P = 0.045) in comparison with urgently operated patients. There was no mortality. Review of the literature (n = 97) showed that crisis patients who underwent elective or urgent surgery vs emergency surgery had less intraoperative (13 of 31 [42%] vs 20 of 25 [80%], P < 0.001) and postoperative complications (15 of 45 [33%] vs 15 of 21 [71%], P = 0.047) and a lower mortality (0 of 64 vs 6 of 33 [18%], P = 0.002).Conclusions:Management of patients presenting with pheochromocytoma crisis should include initial stabilization of the acute crisis followed by sufficient α-blockade before surgery. Emergency resection of pheochromocytoma is associated with high surgical morbidity and mortality.

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    • "On the other hand, α-blockade is the first choice for the management of pheochromocytoma crisis [6] and to reduce the risk of hypertensive crises in patients with biochemically active pheochromocytoma [7]. α-Blockade should be started before β-blockade because stimulation of α-adrenoceptors unopposed by β-adrenoceptor vasodilation can result in hypertensive crises [6] [7]. Vasculitis is a well-described but rare autoimmune complication of pheochromocytoma, triggered by an excess of cathecolamine levels [8]. "

    Full-text · Article · Apr 2014 · American Journal of Emergency Medicine
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    • "60 The mortality from phaeochromocytoma multisystem crisis was reported as 45% after the review of 11 published cases from 1988–2009. 61 Scholten et al. (2013) 6 reported a 0% mortality in their series of 25 cases of PCC, and a 2% (1 of 58) mortality from a literature review they conducted (1990–2013). However, in both instances, patients were only selected if they had proceeded to surgery, and so their cohorts would not have included any patient who died prior to surgery. "
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    ABSTRACT: Pheochromocytoma crisis is an endocrine emergency associated with significant mortality. There is little published guidance on the management of pheochromocytoma crisis. This clinical practice update summarises the relevant published literature, including a detailed review of cases published in the past five years, and a proposed classification system. We review the recommended management of pheochromocytoma crisis including the use of alpha blockade, which is strongly associated with survival of a crisis. Mechanical circulatory supportive therapy (such as intra-aortic balloon pump or extra corporeal membrane oxygenation) is strongly recommended for patients with sustained hypotension. Surgical intervention should be deferred until medical stabilisation is achieved. This article is protected by copyright. All rights reserved.
    Full-text · Article · Sep 2013 · Clinical Endocrinology
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    ABSTRACT: Objectives. To evaluate efficacy of diagnostics and surgical treatment of pheochromocytomas among patients with incidental adrenal mass. Methods. Within the prospective cohort study 322 patients with incidental adrenal mass have been investigated. Indications for surgery were defined to 169 patients. Pheochromocytoma (adrenal paraganglioma) was hystlogically verified in 15 patients. The effectiveness of clinical, laboratory and radiological methods of diagnosis of pheochromocytomas, intraoperative complications, the results of surgical treatment have been analyzed. Results. Combination of arterial hypertension, tachycardia, hyperhidrosis and pallor among clinical signs of hypercatecholamine was considered as the highest diagnostics value (sensitivity 84%, specificity 86%). Multispiral computer tomography (MCT) with contrast is highly sensitive (96%) but low specific (56%) method in the differential diagnostics of pheochromocyte. Diagnostic value of determination of the catecholamine excretion with the urine turned out to be low (sensitivity - 66% and specificity - 96%). The test for dayly metanephrine urine had a great diagnostic significance (sensitivity - 96% and specificity - 100%). Despite on all pheochromocytomas were initially detected as incidentalomas, only 4 (26,6%) cases were referred as silent pheochromocytomas. Intraoperative catecholamine crisis developed in 3 (20%) patients undergoing open surgery and in 1 patient - during laparoscopy. There were no postoperative complications and lethal outcomes. Residual arterial hypertension in long-term period after surgery was detected in 6 (40%) patients. Conclusion. Clinical patterns of hypercatecholaminemia were observed in the majority of patients with adrenal pheochromocytoma previously verified as incidental adrenal mass. The main cause of delay diagnostics of pheochromacytomas was considered as noncompliance by general practitioners of the investigation algorithm of patients observation with arterial hypertension. Silent pheochromocytoma is the most dangerous one which can result as life threaten complication both during examination and surgery. Laparoscopic adrenalectomy is the operation of choice in the case when tumor size > 6 cm.
    Preview · Article · Sep 2013 · Novosti Khirurgii
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