Risk of catecholamine crisis in patients undergoing resection of unsuspected pheochromocytoma

Song, Gina; Joe, Bonnie N.; Yeh, Benjamin M.; Meng, Maxwell V.; Westphalen, Antonio C.; Coakley, Fergus V.

doi:10.1590/s1677-55382011000100005

Cited by 21 publications

(16 citation statements)

References 18 publications

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“…This is accomplished by measuring levels of metanephrine and normetanephrine in plasma or 24-hour urine and mainly serves to prevent unexpected complications during surgery or treatment (96).…”

Section: A Biochemistrymentioning

confidence: 99%

Adrenocortical Carcinoma

et al. 2013

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Adrenocortical carcinoma (ACC) is a rare endocrine malignancy, often with an unfavorable prognosis. Here we summarize the knowledge about diagnosis, epidemiology, pathophysiology, and therapy of ACC. Over recent years, multidisciplinary clinics have formed and the first international treatment trials have been conducted. This review focuses on evidence gained from recent basic science and clinical research and provides perspectives from the experience of a large multidisciplinary clinic dedicated to the care of patients with ACC.

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Section: A Biochemistrymentioning

confidence: 99%

Adrenocortical Carcinoma

et al. 2013

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“…Biochemical evaluation, which is in part guided by hormone-related clinical symptoms of patients, is performed by measurement of steroid hormones potentially produced by the tumor. For several reasons it is important to perform biochemical evaluation prior to surgery (Nieman 2010): i) it can further add to judge the risk of malignancy, since this risk increases in case of androgen or estrogen production; ii) in case of glucocorticoid excess cortisol lowering-or antagonizing therapy can be indicated; iii) patients with cortisol producing ACCs need hydrocortisone replacement post-surgery; iv) hormonal parameters can be used as tumor markers; v) pre-surgical testing for pheochromocytoma-related hormones can avoid complications during surgery (Song et al 2011).…”

Section: Current Tools To Diagnose Accsmentioning

confidence: 99%

Future directions in the diagnosis and medical treatment of adrenocortical carcinoma

Creemers¹,

Hofland²,

Korpershoek³

et al. 2015

Endocrine-Related Cancer

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Adrenocortical carcinoma (ACC) is a rare disease with a poor prognosis. Discrimination between ACCs and adrenocortical adenomas (ACAs) remains challenging, with the current gold standard being the Weiss score, consisting of several histopathological characteristics. However, new markers like Ki67, a marker for proliferation, and the staining of reticulins are promising not only as it comes to identifying malignancy but also as prognostic markers in patients with ACC. Currently, surgery is still the only curative treatment for ACC. Mitotane, an adrenolytic drug, is used in the adjuvant setting and in case of metastatic or advanced disease. Patients with progressive disease are frequently treated with mitotane, alone or in combination with etoposide, doxorubicine and cisplatin. Radiotherapy is indicated in selected cases. The low response rates and high toxicity of the systemic therapies emphasize the need for markers that enable the identification of responders and non-responders. Consequently, research is focusing on predictive factors varying from the expression of DNA repair genes to clinical patient characteristics. Subgroups of ACC with different prognosis have been identified based on transcriptome characteristics. As a conclusion from large molecular studies, ACCs appear to harbor many abnormalities compared to ACAs. Altered pathways driving ACC pathogenesis include the IGF, TP53 and the Wnt signaling pathway, allowing these as new potential targets for medical therapy. However, despite efforts in preclinical and clinical studies investigating efficacy of targeting these pathways, most novel therapies appear to be effective in only a subset of patients with ACC. New treatment concepts are therefore urgently needed.

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“…A subclinical picture does not exclude the potential occurrence of hypertensive crises (62, 63) causing serious cardiovascular events and even the death of the patient. The literature is rich with case reports showing the dramatic consequences of undiagnosed Phaeo/sPGLs and as suggested by the various autopsy studies (15, 64, 65), most of these undiagnosed tumours presumably contribute to premature death.…”

Section: Consequences Of Undiagnosed Subclinical Phaeo/spglmentioning

confidence: 99%

Subclinical phaeochromocytoma

Mannelli

Lenders

Pacák

et al. 2012

Best Practice & Research Clinical Endocrinology & Metab

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Phaeochromocytomas and paragangliomas are neural crest-derived tumours. Autopsy studies indicate that relatively large numbers of these tumours remain undiagnosed during life. This may reflect non-specific signs and symptoms and low medical alertness in evaluating the clinical picture or it may reflect a silent clinical presentation - the subclinical phaeochromocytoma. The associated clinical picture depends on the capacity of the tumours to release catecholamines and sometimes biologically active peptides. Hypertension is the hallmark of catecholamine release, but the amount, type and pattern of catecholamine secretion is extremely variable. Some tumours have low or intermittent secretory activity, some produce mainly or solely dopamine, while others very rarely do not synthesize or release any catecholamines (non-secretory or non-functional tumours). Such tumours may present with mild or even absent signs and symptoms of catecholamine excess. Low secretory activity may reflect small tumour size or differences in secretory phenotypes associated with the biochemical and genetic background of the tumours. Tumours due to succinate dehydrogenase subunit B mutations are often subclinical, poorly differentiated, contain low amounts of catecholamines, and are usually malignant at diagnosis. Adrenoceptor desensitization can result in a subclinical presentation, even when catecholamine levels are high. Subclinical phaeochromocytomas are often discovered as incidentalomas during radiological procedures or during routine screening for phaeochromocytoma in carriers of mutations in one of the ten currently identified tumour susceptibility genes. Undiagnosed phaeochromocytomas, whether or not subclinical and even if biologically benign, may cause extremely deleterious consequences or even death, following abrupt release of catecholamines.

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Risk of catecholamine crisis in patients undergoing resection of unsuspected pheochromocytoma

Cited by 21 publications

References 18 publications

Adrenocortical Carcinoma

Adrenocortical Carcinoma

Future directions in the diagnosis and medical treatment of adrenocortical carcinoma

Subclinical phaeochromocytoma

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