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: 6.31). 01/2013; 98(2). DOI: 10.1210/jc.2012-3020
Source: PubMed

ABSTRACT 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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    ABSTRACT: Objective: The aim was to formulate clinical practice guidelines for pheochromocytoma and paraganglioma (PPGL). Participants: The Task Force included a chair selected by the Endocrine Society Clinical Guidelines Subcommittee (CGS), seven experts in the field, and a methodologist. The authors received no corporate funding or remuneration. Evidence: This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe both the strength of recommendations and the quality of evidence. The Task Force reviewed primary evidence and commissioned two additional systematic reviews. Consensus Process: One group meeting, several conference calls, and e-mail communications enabled consensus. Committees and members of the Endocrine Society, European Society of Endocrinology, and Americal Association for Clinical Chemistry reviewed drafts of the guidelines. Conclusions: The Task Force recommends that initial biochemical testing for PPGLs should include measurements of plasma free or urinary fractionated metanephrines. Consideration should be given to preanalytical factors leading to false-positive or false-negative results. All positive results require follow-up. Computed tomography is suggested for initial imaging, but magnetic resonance is a better option in patients with metastatic disease or when radiation exposure must be limited. (123)I-metaiodobenzylguanidine scintigraphy is a useful imaging modality for metastatic PPGLs. We recommend consideration of genetic testing in all patients, with testing by accredited laboratories. Patients with paraganglioma should be tested for SDHx mutations, and those with metastatic disease for SDHB mutations. All patients with functional PPGLs should undergo preoperative blockade to prevent perioperative complications. Preparation should include a high-sodium diet and fluid intake to prevent postoperative hypotension. We recommend minimally invasive adrenalectomy for most pheochromocytomas with open resection for most paragangliomas. Partial adrenalectomy is an option for selected patients. Lifelong follow-up is suggested to detect recurrent or metastatic disease. We suggest personalized management with evaluation and treatment by multidisciplinary teams with appropriate expertise to ensure favorable outcomes.
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    ABSTRACT: Case report: Authors present the case of a 30-year-old female patient, who was admitted to the ICU because of hypertensive crisis accompanied by chest complains, cardiac decompensation, progrediating short of breath and unconsciousness. Despite the quick examinations and the prompt treatment multi-organ failure developed 3 days after admission. Investigations revealed the underlying cause, which was a left-sided suprarenal neoplasm. Hence, multidisciplinary decision was made to carry out a laparoscopic adrenalectomy urgently. The histology examination of the removed neoplasm was pheochromocytoma. In the postoperative period the condition of the patient gradually improved, her symptoms and complains settled, and finally she was discharged in a healthy condition. Discussion: The diagnosis of a pheochromocytoma is a difficult task, the symptoms and complains caused by it can simulate many other illnesses. The acute crisis caused by pheochromocytoma usually can be treated conservatively, but in more severe cases with impending multi-organ failure an urgent operative treatment can be unavoidable. Though the operative risk is relatively high, the correct intra- and postoperative treatment with a quick laparoscopic procedure can be effective.
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    Der Internist 10/2013; 54(10). DOI:10.1007/s00108-013-3293-9 · 0.27 Impact Factor