Malignant Pheochromocytoma

Rao, Fangwen; Keiser, Harry R.; O’Connor, Daniel T.

doi:10.1161/01.hyp.36.6.1045

Cited by 89 publications

(24 citation statements)

References 31 publications

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“…A Chrisoulidou et al: Malignant phaeochromocytoma and paraganglioma www.endocrinology-journals.org chromaffin-cell tumours associated with MEN 2 syndrome usually produce both adrenaline and noradrenaline (Rao et al 2000, Eisenhofer et al 2001, van der Harst et al 2002. Occasionally, malignant tumours can secrete preferentially dopamine (Proye et al 1986, Brouwers et al 2006a due to alterations of catecholamine synthesis in malignant phaeochromocytoma cells (John et al 1999).…”

Section: Biochemical Diagnosis Of Malignant Chromaffin-cell Tumoursmentioning

confidence: 99%

“…The CgA expression is present in both benign and malignant phaeochromocytomas, although different patterns of expression exist in malignant tumours (Portel-Gomes et al 2001). The CgA is elevated in both functioning and nonfunctioning chromaffin-cell tumours (Guignat et al 2001), whereas markedly increased levels may be indicative of a malignant tumour in a small series of patients (Rao et al 2000). Besides its diagnostic significance, the CgA can also be used to monitor response to treatment and/or indicate relapse of the disease (Grossrubatscher et al 2006).…”

Section: Biochemical Diagnosis Of Malignant Chromaffin-cell Tumoursmentioning

confidence: 99%

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The diagnosis and management of malignant phaeochromocytoma and paraganglioma

Chrisoulidou

Kaltsas²,

Ilias³

et al. 2007

Endocr Relat Cancer

293

219

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Malignant phaeochromocytomas are rare tumours accounting for w10% of all phaeochromocytomas; the prevalence of malignancy among paragangliomas is higher, especially those associated with succinate dehydrogenase subunit B gene mutations. Although a subset of these tumours has metastatic disease at initial presentation, a significant number develops metastases during follow-up after excision of an apparently benign tumour. Clinical, biochemical and histological features cannot reliably distinguish malignant from benign tumours. Although a number of recently introduced molecular markers have been explored, their clinical significance remains to be elucidated from further studies. Several imaging modalities have been utilised for the diagnosis and staging of these tumours. Functional imaging using radiolabelled metaiodobenzylguanidine (MIBG) and more recently, 18 F-fluorodopamine and 18 F-fluorodopa positron emission tomography offer substantial sensitivity and specificity to correctly detect metastatic phaeochromocytoma and paraganglioma and helps identify patients suitable for treatment with radiopharmaceuticals. The 5-year mortality rate of patients with malignant phaeochromocytomas and paragangliomas greater than 50% indicates that there is considerable room for the improvement of currently available therapies. The main therapeutic target is tumour reduction and control of symptoms of excessive catecholamine secretion. Currently, the best adjunctive therapy to surgery is treatment with radiopharmaceuticals using 131 I-MIBG; however, this is very rarely curative. Chemotherapy has been used for metastatic disease with only a partial and mainly palliative effect. The role of other forms of radionuclide treatment either alone or in combination with chemotherapy is currently evolving. Ongoing microarray studies may provide novel intracellular pathways of importance for proliferation/cell cycle control, and lead to the development of novel pharmacological agents.

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Section: Biochemical Diagnosis Of Malignant Chromaffin-cell Tumoursmentioning

confidence: 99%

Section: Biochemical Diagnosis Of Malignant Chromaffin-cell Tumoursmentioning

confidence: 99%

The diagnosis and management of malignant phaeochromocytoma and paraganglioma

Chrisoulidou

Kaltsas²,

Ilias³

et al. 2007

Endocr Relat Cancer

293

219

View full text Add to dashboard Cite

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“…The commonalities of gene expression in malignant and VHL-associated pheochromocytomas appear instead to reflect the biochemical phenotype common to these tumors. Malignant and VHL-associated pheochromocytomas produce predominantly norepinephrine and usually have an exclusively noradrenergic biochemical phenotype, whereas MEN 2 tumors produce both epinephrine and norepinephrine (Rao et al 2000, Eisenhofer et al 2001, van der Harst et al 2002. Sporadic, VHL, and malignant pheochromocytomas with a noradrenergic phenotype all show increased expression of the gene for endothelial Per-Arylhydrocarbon receptor nuclear translocator-Sim (PAS) domain protein 1 (Hif-2a) compared with benign hereditary and sporadic tumors that (Walther et al 1999;Aguiar et al 2001;Neumann et al 2002;Bryant et al 2003;Bauters et al 2003;Gimenez-Roqueplo et al 2003).…”

Section: Molecular Geneticsmentioning

confidence: 99%

Malignant pheochromocytoma: current status and initiatives for future progress

Eisenhofer

Bornstein

Brouwers

et al. 2004

Endocr Relat Cancer

305

226

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Pheochromocytomas are rare catecholamine-producing neuroendocrine tumors that are usually benign, but which may also present as or develop into a malignancy. Predicting such behavior is notoriously difficult and there are currently no curative treatments for malignant tumors. This report follows from a workshop at the Banbury Conference Center, Cold Spring Harbor, New York, on the 16th-18th November 2003, held to review the state of science and to facilitate future progress in the diagnosis and treatment of malignant pheochromocytoma. The rarity of the tumor and the resulting fragmented nature of studies, typically involving small numbers of patients, represent limiting factors to the development of effective treatments and diagnostic or prognostic markers for malignant disease. Such development is being facilitated by the availability of new genomics-based tools, but for such approaches to succeed ultimately requires comprehensive clinical studies involving large numbers of patients, stringently collected clinical data and tumor samples, and interdisciplinary collaborations among multiple specialist centers. Nevertheless, the well-characterized hereditary basis and the unique functional nature of these neuroendocrine tumors provide a useful framework that offers advantages for establishing the pathways of tumorigenesis and malignancy. Such findings may have relevance for understanding the basis of other more common malignancies where similar frameworks are not available. As the relevant pathways leading to pheochromocytoma are established it should be possible to take advantage of the new generation of drugs being developed to target specific pathways in other malignancies. Again the success of this will require well-designed and coordinated multicenter studies.

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“…Plasma levels of chromogranin A (CgA), secreted from neurosecretory vesicles along with catecholamines (33), are often elevated in both functioning and silent PGLs and particularly in malignant ones (34). CgA levels correlate with tumor mass, making it a useful tumor marker (35).…”

Section: Sympathetic Pgl/pccmentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Clinical management of paragangliomas

Corssmit

Romijn

2014

European Journal of Endocrinology

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Paragangliomas (PGLs) are rare vascular, neuroendocrine tumors of paraganglia, which are associated with either sympathetic tissue in adrenal (pheochromocytomas (PCCs)) and extraadrenal (sympathetic paraganglioma (sPGLs)) locations or parasympathetic tissue of the head and neck paragangliomas (HNPGLs). As HNPGLs are usually benign and most tumors grow slowly, a wait-and-scan policy is often advised. However, their location in the close proximity to cranial nerves and vasculature may result in considerable morbidity due to compression or infiltration of the adjacent structures, necessitating balanced decisions between a wait-and-see policy and active treatment. The main treatment options for HNPGL are surgery and radiotherapy. In contrast to HNPGLs, the majority of sPGL/PCCs produces catecholamines, in advanced cases resulting in typical symptoms and signs such as palpitations, headache, diaphoresis, and hypertension. The state-of-the-art diagnosis and localization of sPGL/PCCs are based on measurement of plasma and/or 24-h urinary excretion of (fractionated) metanephrines and methoxytyramine (MT). sPGL/PCCs can subsequently be localized by anatomical (computed tomography and/or magnetic resonance imaging) and functional imaging studies ( 123 I-metaiodobenzylguanidine-scintigraphy, 111 In-pentetreotide scintigraphy, or positron emission tomography with radiolabeled dopamine or dihydroxyphenylalanine). Although most PGL/PCCs are benign, factors such as genetic background, tumor size, tumor location, and high MT levels are associated with higher rates of metastatic disease. Surgery is the only curative treatment. Treatment options for patients with metastatic disease are limited. PGL/PCCs have a strong genetic background, with at least one-third of all cases linked with germline mutations in 11 susceptibility genes. As genetic testing becomes more widely available, the diagnosis of PGL/PCCs will be made earlier due to routine screening of at-risk patients. Early detection of a familial PGL allows early detection of potentially malignant PGLs and early surgical treatment, reducing the complication rates of this operation.

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Malignant Pheochromocytoma

Cited by 89 publications

References 31 publications

The diagnosis and management of malignant phaeochromocytoma and paraganglioma

The diagnosis and management of malignant phaeochromocytoma and paraganglioma

Malignant pheochromocytoma: current status and initiatives for future progress

MANAGEMENT OF ENDOCRINE DISEASE: Clinical management of paragangliomas

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