Clinical Manifestations

Manger, William M.; Gifford, Ray W.

doi:10.1007/978-1-4612-9900-4_4

Cited by 11 publications

(26 citation statements)

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“…Von Euler and Holtz independently reported the occurrence of norepinephrine in the human body (Holtz et al, 1947; von Euler, 1946a, 1946b). The first successful surgical removal of pheochromocytoma was performed by Roux in 1926 (Manger and Gifford, 1996). …”

Section: Pastmentioning

confidence: 99%

Pheochromocytoma and Paraganglioma

Kantorovich

Pacák

2010

Neuroendocrinology - Pathological Situations and Diseases

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Pheochromocytoma is a very special kind of tumor full of duplicity. On the one hand it represents its own microworld with unique clinical, biochemical and pathological features, while on the other it constitutes a tremendously significant part of whole body system, playing a vital role for practically every organ system. It has a very special character – sometimes like a child it can be sweet and predictable, while at times it can behave like a deadly wild beast, crashing and tearing everything on its path in a fierce rage. It also consists of the amazingly intelligent neuroendocrine cells that possess a magical ability to make miraculous substances of many kinds. But most of all, it is a system that is able to drive our curiosity and the itch of “Cogito, ergo sum” to limitless depths and year by year it still amazes us with new and unexpected discoveries that move our understanding of multiple pathways and metabolic events closer to the ultimate truth. Recent discoveries of succinate dehydrogenase (SHD) and prolyl hydroxylase (PHD) mutations, for example, propelled our understanding of neuroendocrine tumorigenesis as a whole, as well as physiology of mitochondrial respiratory chain and phenomenon of pseudohypoxia in particular. Good old discoveries make their way from dusty repositories to shine with new meaning, appropriate for the current level of knowledge. This acquired wisdom makes us better physicians – knowing the specific expression makeup of catecholamine transporters, GLUTs and SRIFs allows for better tailored imaging and therapeutic manipulations. There are still long ways to go, keeping in mind that pheochromocytoma is but so very special, and we are optimistic and expect many great things to come.

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Section: Pastmentioning

confidence: 99%

Pheochromocytoma and Paraganglioma

Kantorovich

Pacák

2010

Neuroendocrinology - Pathological Situations and Diseases

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“…The clinical manifestation of pheochromocytoma/paraganglioma is typically characterized by symptoms of elevated blood pressure, heart palpitations, increased perspiration, and anxiety 4–6 . In 2000 a 23-year-old male patient presented to the National Institutes of Health (NIH) for suspected pheochromocytoma/paraganglioma.…”

Section: Introductionmentioning

confidence: 99%

Exercise-induced nausea and vomiting: another sign and symptom of pheochromocytoma and paraganglioma

King

Darmani

Hughes

et al. 2010

Endocr

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Objective A cohort of nine patients, mostly young adults, presented with a new symptom of pheochromocytoma/paraganglioma: exercise induced nausea and vomiting. The aim of this report is to present this symptom of pheochromocytoma/paraganglioma and to suggest a hypothesis for the observation. Design This is a prospective study looking at the patient accounts of the reported symptom and the clinical data on the patients disease. Methods Following a 2000 report from a paraganglioma patient of the experience of exercise induced nausea and vomiting, researchers and clinicians at the National Institutes of Health (NIH), pheochromocytoma/paraganglioma protocol, began asking patients about instances of nausea and vomiting induced through exercise. A cohort of nine patients reporting exercise induced nausea and vomiting was found and their clinical data are analyzed and presented here. Results Exercise induced nausea and vomiting in pheochromocytoma/paraganglioma patients is most likely due to the elevation of circulating catecholamines that activate adrenergic receptors of the area postrema, which induces nausea and vomiting. Succinate dehydrogenase subunit B mutations/deletions were prevalent in the patients described. Conclusions Post exercise nausea and vomiting should be considered as a symptom of pheochromocytoma/paraganglioma and should be addressed in the clinical evaluation of these patients, especially in young adults.

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“…Pheochromocytomas are tumors arising from chromaffin cells, mainly of the adrenal gland, that synthesize, store, metabolize, and usually but not always secrete catecholamines (Manger & Gifford 1996). Most are benign and curable by surgical resection, but some are clinically malignant (Lehnert et al 2004).…”

Section: Introductionmentioning

confidence: 99%

Malignant pheochromocytoma: current status and initiatives for future progress

Eisenhofer

Bornstein

Brouwers

et al. 2004

Endocr Relat Cancer

303

218

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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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Clinical Manifestations

Cited by 11 publications

References 0 publications

Pheochromocytoma and Paraganglioma

Pheochromocytoma and Paraganglioma

Exercise-induced nausea and vomiting: another sign and symptom of pheochromocytoma and paraganglioma

Malignant pheochromocytoma: current status and initiatives for future progress

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