Preoperative Management of the Pheochromocytoma Patient

Pacák, Karel

doi:10.1210/jc.2007-1720

Cited by 515 publications

(503 citation statements)

References 65 publications

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“…Untreated pheochromocytomas can result in hypertension and subsequent acute heart disease, brain edema, and stroke. Familial pheochromocytomas are often a result of mutations in VHL (MIM]s 193300, 608537), Neurofibromatosis-1 (NF1), c-RET (mutated in Multiple Endocrine Neoplasia-2), or succinate dehydrogenase (SDHB, SDHC, SDHD) genes [Astuti et al, 2001;Cascó n et al, 2009;Lee et al, 2005;Neumann et al, 2002;Pacak, 2007]. However, true sporadic pheochromocytomas rarely have somatic mutations in these genes.…”

Section: Introductionmentioning

confidence: 99%

Genetic analysis of von Hippel-Lindau disease

et al. 2010

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Mutations in the von Hippel-Lindau (VHL) gene are responsible for VHL disease, congenital polycythemia, and are found in many sporadic tumor types as well. Reports of VHL mutations are dispersed throughout original articles and databases that have not been recently updated. We compiled a comprehensive mutation table of 1,548 germline and somatic VHL mutations, derived from this protein of only 213 amino acids. We describe detailed phenotype and gene mutation information for 945 VHL families, including 30 previously unpublished kindreds from The Netherlands (six novel mutations). These data represent the most extensive catalog of germline VHL mutations to date. We also review VHL disease, known and theorized pathogenesis of common VHL manifestations, and genotype-phenotype correlations. Analysis of all VHL families, excluding germline mutations resulting in congenital polycythemias, describes the spectrum of mutation types: 52% missense, 13% frameshift, 11% nonsense, 6% in-frame deletions/insertions, 11% large/complete deletions, and 7% splice mutations. This easy-to-use compilation of VHL mutations is intended to facilitate research and function as a necessary adjunct for physicians when providing patient information. Hum Mutat 31:521-537,

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Section: Introductionmentioning

confidence: 99%

Genetic analysis of von Hippel-Lindau disease

et al. 2010

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“…When these hormones are released, epinephrine acts on alpha and beta adrenergic receptors while norepinephrine acts on the same receptors, except b2 adrenergic receptors. The cumulative effect is potent peripheral vasoconstriction by alpha receptor agonism and increased heart rate by b1 agonism 6 . Thus, management of hypertension due to a secondary cause like pheochromocytoma is very specific.…”

Section: Discussionmentioning

confidence: 99%

“…The rationale is that beta-blockade alone would result in blocking of beta receptors that cause peripheral vasodilation, leaving alpha mediated peripheral vasoconstriciton unopposed. Additionally, Metyrosine, a competitive inhibitor of the enzyme needed for catecholamine synthesis, has been proposed for pheochromocytoma management but is rarely clinically utilized at this time 6 .…”

Section: Discussionmentioning

confidence: 99%

A Case Report of a Pheochromocytoma Presenting With Neurological Manifestations

Shamsuddin¹,

Jackson²

2009

TMF

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A 51 year-old Caucasian female with a past medical history of hypertension, coronary artery disease and cerebrovascular accident presented to the emergency room with acute onset dysarthria and right-sided hemiparesis. The patient reported that she noticed these symptoms when she woke that morning, but had resolved by the time she reached the emergency room, an hour later. She denied experiencing palpitations, headache, chest pain or shortness of breath.On review of systems, it was revealed she had intermittent nausea and vomiting for a year with increasing frequency over the past two days. She denied blood or bile in the emesis. She also admitted to a 50-pound weight loss over the past year. Both esophagogastroduodenoscopy and colonoscopy were normal within the past year.Her medical history was significant for coronary artery disease complicated by two myocardial infarctions the previous year. She had also suffered from two past cerebrovascular accidents -the second of which was complicated by hemorrhage and seizure. Despite several anti-hypertensive medications, the patient's blood pressure remained uncontrolled.On admission, the patient was afebrile, had an elevated blood pressure of 167/108, pulse of 95 beats/minute, respiratory rate of 18 breathes/minute, and pulse oximetry of 97% on room air. Generally, the patient appeared comfortable. Cardiovascular exam was benign and pulmonary exam revealed decreased breath sounds bilaterally. Abdominal exam was also benign with no masses appreciated. Neurological exam was significant for slight dysarthria, right homonymous hemianopsia, and right-sided facial droop. Upper and lower extremity strength testing was significant for weakness at 4/5 strength on the left side. Comparatively, the patient's strength was 5/5 in the upper and lower extremities on the right side. Additionally, she was hyper-reflexive on the right side compared to the left. Sensation was intact throughout. These neurological deficits were consistent with her baseline deficits from past strokes.CT of the head was significant for an old left middle cerebral artery stroke, but there was no evidence of an acute process. A CT of the abdomen was performed in the emergency room, which revealed an incidental finding of a 6.7 x 5.4 cm right sided adrenal mass (Figure 1).The laboratory work-up revealed elevated catecholamines: plasma norepinephrine of 41.6, plasma metanephrine of 27.2, fractionated urine norepinephrine of 9390 and fractionated urine metanephrine of 8231.Diagnoses of transient ischemic attack and pheochromocytoma were made. Her blood pressure medications were changed to the alpha-blocker, phenoxybenzamine. A beta-blocker was later added for additional blood pressure control. The mass was surgically removed four weeks later. DiscussionA pheochromocytoma is a tumor that results in excess secretion of the catecholamines epinephrine and norepinephrine. It arises from chromaffin cells of the medulla of the adrenal gland, but it can also be located in extra-adrenal, retroperitoneal, pelvic or thorac...

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“…69 The detected fibrosis was at times focal and at times diffuse. 14 The detected myocardial dysfunction was not only both systolic and diastolic, but did not always readily resolve after tumor removal. 69 Furthermore, it should be noted that the cardiac phenotype in pheochromocytoma frequently can resemble Takotsubo cardiomyopathy with left ventricular regional wall motion abnormalities.…”

Section: Echocardiography In the Management Of Pheochromocytomamentioning

confidence: 95%

“…57 The primary assumption of this commentary's author would have been "too-early extubation in a patient with extended atelectasis related to a long surgical procedure." 58 Because a chest x-ray was reported to show pulmonary edema, at least the following 3 additional diagnoses should have been discussed: (1) nonspecific respiratory distress syndrome, 14 (2) lung injury directly related to blood transfusion, 59 and (3) Takotsubo-like myocardial injury resulting from intraoperative catecholamine release. 30 In like manner to that previously indicated, prolonged hypercarbia made the diagnosis of Takotsubo-like cardiomyopathy very unlikely.…”

Section: Specific Comments On the Reported Respiratory Failure Are Wamentioning

confidence: 99%