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Clinical Medicine (London, England) Jan 2019
Review
Topics: Adrenal Gland Neoplasms; Humans; Pheochromocytoma
PubMed: 30651249
DOI: 10.7861/clinmedicine.19-1-68 -
Journal of Clinical Hypertension... 2002Pheochromocytoma, a relatively rare (<0.05% of hypertensives), catecholamine-secreting tumor, is almost always lethal unless recognized and appropriately treated.... (Review)
Review
Pheochromocytoma, a relatively rare (<0.05% of hypertensives), catecholamine-secreting tumor, is almost always lethal unless recognized and appropriately treated. Clinical and biochemical manifestations are mainly caused by excess circulating catecholamines and hypertension. Manifestations mimic many conditions, which may result in erroneous diagnoses and improper treatment. Sustained or paroxysmal hypertension associated with headaches, sweating, or palpitations, occurs in 95% of patients, but at least 5% are normotensive. All patients with manifestations of hypercatecholaminemia or coexisting neoplasms should be investigated for pheochromocytoma. Plasma free metanephrines and fractionated urinary metanephrines are the most sensitive (about 100%) chemical tests for diagnosing sporadic and familial pheochromocytomas; plasma and urinary catecholamines and total metanephrines are fairly sensitive for identifying sporadic cases but are less sensitive for familial tumors. The clonidine suppression test helps exclude other conditions that may elevate plasma and urinary catecholamines and their metabolites. Magnetic resonance imaging is more sensitive than computed tomography for localizing pheochromocytomas; iodine-131-metaiodobenzylguanidine (131I-MIBG) tumor uptake confers specificity. Surgical resection is successful in 90% of cases, but 10% of tumors are malignant. Pheochromocytomas <5 cm in diameter can be removed laparoscopically; larger tumors should be removed by open surgery. Drug treatment prior to and during surgery is mandatory; drug treatment, chemotherapy, and radiation therapy are used to treat malignant lesions.
Topics: Adrenal Gland Neoplasms; Algorithms; Diagnosis, Differential; Humans; Hypertension; Pheochromocytoma
PubMed: 11821644
DOI: 10.1111/j.1524-6175.2002.01452.x -
Clinical Chemistry Mar 2013
Topics: Adrenal Gland Neoplasms; Diagnosis, Differential; Genetic Predisposition to Disease; Genetic Testing; Humans; Pheochromocytoma; Prevalence
PubMed: 23209036
DOI: 10.1373/clinchem.2012.182246 -
Clinical & Translational Oncology :... Jul 2021The subclassification of adrenal cancers according to the WHO classification in ordinary, myxoid, oncocytic, and sarcomatoid as well as pediatric types is well...
PURPOSE
The subclassification of adrenal cancers according to the WHO classification in ordinary, myxoid, oncocytic, and sarcomatoid as well as pediatric types is well established, but the criteria for each subtype are not sufficiently determined and the relative frequency of the different types of adrenal cancers has not been studied in large cohorts. Therefore, our large collection of surgically removed adrenal cancers should be reviewed o establish the criteria for the subtypes and to find out the frequency of the various types.
METHODS
In our series of 521 adrenal cancers the scoring systems of Weiss et al., Hough et al., van Slooten et al. and the new Helsinki score system were used for the ordinary type of cancer (97% of our series) and the myxoid type (0.8%). For oncocytic carcinomas (2%), the scoring system of Bisceglia et al. was applied.
RESULTS
Discrepancies between benign and malignant diagnoses from the first thee classical scoring systems are not rare (22% in our series) and could be resolved by the Helsinki score especially by Ki-67 index (more than 8% unequivocally malignant). Since all our cancer cases are positive in the Helsinki score, this system can replace the three elder systems. For identification of sarcomatoid cancer as rarest type in our series (0.2%), the scoring systems are not practical but additional immunostainings used for soft tissue tumors and in special cases molecular pathology are necessary to differentiate these cancers from adrenal sarcomas. According to the relative frequencies of the different subtypes of adrenal cancers the main type is the far most frequent (97%) followed by the oncocytic type (2%), the myxoid type (0.8%) and the very rare sarcomatoid type (0.2%).
CONCLUSIONS
The Helsinki score is the best for differentiating adrenal carcinomas of the main, the oncocytic, and the myxoid type in routine work. Additional scoring systems for these carcinomas are generally not any longer necessary. Signs of proliferation (mitoses and Ki-67 index) and necroses are the most important criteria for diagnosis of malignancy.
Topics: Adrenal Gland Neoplasms; Humans; Neoplasm Grading
PubMed: 33818702
DOI: 10.1007/s12094-020-02524-2 -
Australian Journal of General Practice 2021Diseases of the adrenal gland occur rather more frequently than is appreciated and provide a series of challenges for the treating practitioner.
BACKGROUND
Diseases of the adrenal gland occur rather more frequently than is appreciated and provide a series of challenges for the treating practitioner.
OBJECTIVE
The aim of this article is to provide a practical approach to common adrenal disorders encountered in general practice, including adrenal incidentalomas, primary aldosteronism and adrenal insufficiency.
DISCUSSION
Adrenal incidentalomas are adrenal mass lesions >1 cm in diameter serendipitously discovered by radiological examination. They require structural assessment to distinguish common benign pathologies from the rare malignant ones, and biochemistry to exclude hypersecretion syndromes resulting from excess cortisol, aldosterone or catecholamines. Primary aldosteronism represents >5% of hypertension and may be cured or specifically treated, yet is rarely screened for in primary care. Low cortisol levels may reflect adrenal insufficiency due to either adrenal failure, where adrenocorticotropic hormone levels will be elevated, or secondary to hypothalamic-pituitary dysfunction resulting from structural lesions or, increasingly, prescribed exogenous synthetic glucocorticoids and nonconventional therapies.
Topics: Adrenal Gland Neoplasms; Adrenal Glands; Aldosterone; Humans; Hydrocortisone; Hyperaldosteronism
PubMed: 33543156
DOI: 10.31128/AJGP-09-20-5619 -
Cancer Imaging : the Official... Mar 2010With the increasing use of abdominal cross-sectional imaging, incidental adrenal masses are being detected more often. The important clinical question is whether these... (Review)
Review
With the increasing use of abdominal cross-sectional imaging, incidental adrenal masses are being detected more often. The important clinical question is whether these lesions are benign adenomas or malignant primary or secondary masses. Benign adrenal masses such as lipid-rich adenomas, myelolipomas, adrenal cysts and adrenal haemorrhage have pathognomonic cross-sectional imaging appearances. However, there remains a significant overlap between imaging features of some lipid-poor adenomas and malignant lesions. The nature of incidentally detected adrenal masses can be determined with a high degree of accuracy using computed tomography (CT) and magnetic resonance imaging (MRI) alone. Positron emission tomography (PET) is also increasingly used in clinical practice in characterizing incidentally detected lesions. We review the performance of the established and new techniques in CT, MRI and PET that can be used to distinguish benign adenomas and malignant lesions of the adrenal gland.
Topics: Adenoma; Adrenal Gland Neoplasms; Adrenal Glands; Adult; Biopsy; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Positron-Emission Tomography; Tomography, X-Ray Computed
PubMed: 20299300
DOI: 10.1102/1470-7330.2010.0012 -
Journal of Visceral Surgery Oct 2014Improvements in medical imaging have resulted in the incidental discovery of many silent and unrecognized adrenal tumors. The term "adrenal incidentaloma" (AI) is... (Review)
Review
Improvements in medical imaging have resulted in the incidental discovery of many silent and unrecognized adrenal tumors. The term "adrenal incidentaloma" (AI) is applied to any adrenal mass≥1cm in its longest axis that is discovered incidentally during abdominal imaging that was not performed to specifically evaluate adrenal pathology. These incidentalomas may be either secretory or non-secretory, benign or malignant. Distinctive characteristics of these lesions must be determined by the clinician to determine appropriate management. Such distinctions are based on laboratory findings and imaging, principally CT with and without contrast injection. Investigations must be carefully chosen to avoid ordering unnecessary and expensive tests for too many patients while, at the same time, avoiding the risk of failing to diagnose a secreting malignant or tumor. These examinations will determine patient care: surgery or surveillance. When simple surveillance is chosen, specific criteria must be met with regard to diagnostic modalities (clinical, imaging, laboratory testing) and its duration.
Topics: 3-Iodobenzylguanidine; Adrenal Gland Neoplasms; Biopsy; Cortisone; Humans; Incidental Findings; Positron-Emission Tomography; Tomography, X-Ray Computed
PubMed: 25127879
DOI: 10.1016/j.jviscsurg.2014.07.002 -
Cancer Medicine Jul 2023Pheochromocytomas (PCCs) are rare neuroendocrine tumors derived from the chromaffin cells of the adrenal medulla. When these tumors have an extra-adrenal location, they... (Review)
Review
Pheochromocytomas (PCCs) are rare neuroendocrine tumors derived from the chromaffin cells of the adrenal medulla. When these tumors have an extra-adrenal location, they are called paragangliomas (PGLs) and arise from sympathetic and parasympathetic ganglia, particularly of the para-aortic location. Up to 25% of PCCs/PGLs are associated with inherited genetic disorders. The majority of PCCs/PGLs exhibit indolent behavior. However, according to their affiliation to molecular clusters based on underlying genetic aberrations, their tumorigenesis, location, clinical symptomatology, and potential to metastasize are heterogenous. Thus, PCCs/PGLs are often associated with diagnostic difficulties. In recent years, extensive research revealed a broad genetic background and multiple signaling pathways leading to tumor development. Along with this, the diagnostic and therapeutic options were also expanded. In this review, we focus on the current knowledge and recent advancements in the diagnosis and treatment of PCCs/PGLs with respect to the underlying gene alterations while also discussing future perspectives in this field.
Topics: Humans; Pheochromocytoma; Paraganglioma; Carcinogenesis; Cell Transformation, Neoplastic; Adrenal Gland Neoplasms
PubMed: 37145019
DOI: 10.1002/cam4.6010 -
Frontiers in Endocrinology 2023Pheochromocytomas and Paragangliomas (Pheo/PGL) are rare catecholamine-producing tumours derived from adrenal medulla or from the extra-adrenal paraganglia respectively.... (Review)
Review
Pheochromocytomas and Paragangliomas (Pheo/PGL) are rare catecholamine-producing tumours derived from adrenal medulla or from the extra-adrenal paraganglia respectively. Around 10-15% of Pheo/PGL develop metastatic forms and have a poor prognosis with a 37% of mortality rate at 5 years. These tumours have a strong genetic determinism, and the presence of succinate dehydrogenase B (SDHB) mutations are highly associated with metastatic forms. To date, no effective treatment is present for metastatic forms. In addition to cancer cells, the tumour microenvironment (TME) is also composed of non-neoplastic cells and non-cellular components, which are essential for tumour initiation and progression in multiple cancers, including Pheo/PGL. This review, for the first time, provides an overview of the roles of TME cells such as cancer-associated fibroblasts (CAFs) and tumour-associated macrophages (TAMs) on Pheo/PGL growth and progression. Moreover, the functions of the non-cellular components of the TME, among which the most representatives are growth factors, extracellular vesicles and extracellular matrix (ECM) are explored. The importance of succinate as an oncometabolite is emerging and since Pheo/PGL SDH mutated accumulate high levels of succinate, the role of succinate and of its receptor (SUCNR1) in the modulation of the carcinogenesis process is also analysed. Further understanding of the mechanism behind the complicated effects of TME on Pheo/PGL growth and spread could suggest novel therapeutic targets for further clinical treatments.
Topics: Humans; Pheochromocytoma; Tumor Microenvironment; Paraganglioma; Adrenal Gland Neoplasms; Succinates
PubMed: 37033265
DOI: 10.3389/fendo.2023.1137456 -
International Journal of Surgery... 2014Pheochromocytoma (PCC), a rare neuroendocrine tumor, shows a prevalence ranging between 0.1% and 0.6% in individuals suffering from hypertension. To date, an increasing... (Review)
Review
Pheochromocytoma (PCC), a rare neuroendocrine tumor, shows a prevalence ranging between 0.1% and 0.6% in individuals suffering from hypertension. To date, an increasing number of patients with hereditary forms or subclinical PCCs have been diagnosed. We reviewed the main controversies and the most recent updates, especially inheritance genetics and surgical management. According to the "rule of 10", in 1/10 patients with pheochromocytoma it is malignant, in 1/10 of cases the tumor is bilateral, in 1/10 extra-adrenal and in 1/10 familial. Surgical resection, the only curative treatment, carries a high risk of hypertensive crises due to massive catecholamine release. Alpha 1 blocker therapy, alone or in combination with beta blockers, calcium antagonists, and plasma volume expansion, is the most commonly used preoperative treatment protocol. Minimally invasive adrenalectomy (laparoscopic and retro-peritoneoscopic) allows earlier mobilization and recovery, reducing the risk of pulmonary infections and thromb-oembolic complications, and is associated with lower morbidity and mortality rates than traditional surgery; it is currently considered the gold standard for the treatment of adrenal tumors ≤6 cm in diameter and weighing < 100 g. Genetic testing will increasingly be the key factor in estimating the life-long risk for development of recurrent disease, contralateral disease or malignant dedifferentiation, thus influencing follow-up protocols.
Topics: Adrenal Gland Neoplasms; Humans; Pheochromocytoma
PubMed: 24727002
DOI: 10.1016/j.ijsu.2014.04.001