Incidental and Metastatic Adrenal Masses

Mannelli, Massimo; Colagrande, Stefano; Valeri, Andrea; Parenti, Gabriele

doi:10.1053/j.seminoncol.2010.10.018

Cited by 18 publications

(13 citation statements)

References 81 publications

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“…Phaeo/sPGL are highly vascularised tumours, often presenting with haemorrhagic areas (17, 24). Therefore, also large Phaeo/sPGL with extensive internal necrotic/haemorrhagic areas may be associated with a mild clinical picture.…”

Section: Tumour Characteristics Influencing the Clinical Picturementioning

confidence: 99%

“…These characteristics account for their typical high signal on T2-weighted imaging and strong enhancement after contrast agent administration (17). Although suggestive, these findings do not allow the diagnosis, which has to be confirmed by laboratory results.…”

Section: Clinical Settings With a Subclinical Phaeo/spglmentioning

confidence: 99%

“…Before 1962, 53% of these tumours were reported to go undiagnosed before surgery or autopsy (14, 15). After the advent of imaging techniques such as sonography, computed tomography (CT) and magnetic resonance imaging (MRI), this percentage has certainly dropped; in fact, at present, a consistent percentage of phaeo/sPGL are detected as incidental masses, mostly in the adrenals (adrenal incidentalomas) (16, 17). Nevertheless, large proportions of these tumours remain undiagnosed or are diagnosed at a late stage after occurrence of cardiovascular complications.…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Tumour Characteristics Influencing the Clinical Picturementioning

confidence: 99%

Section: Clinical Settings With a Subclinical Phaeo/spglmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Subclinical phaeochromocytoma

Mannelli

Lenders

Pacák

et al. 2012

Best Practice & Research Clinical Endocrinology & Metab

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“…Nevertheless, in large tumors with haemorrhagic and/or necrotic areas (features often detected in malignant lesions), the signal intensity on T2-weighted images may be low [23]. …”

Section: Diagnosis: Anatomical and Functional Imagingmentioning

confidence: 99%

Updated and New Perspectives on Diagnosis, Prognosis, and Therapy of Malignant Pheochromocytoma/Paraganglioma

Parenti

Zampetti

Rapizzi

et al. 2012

Journal of Oncology

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112

101

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Malignant pheochromocytomas/paragangliomas are rare tumors with a poor prognosis. Malignancy is diagnosed by the development of metastases as evidenced by recurrences in sites normally devoid of chromaffin tissue. Histopathological, biochemical, molecular and genetic markers offer only information on potential risk of metastatic spread. Large size, extraadrenal location, dopamine secretion, SDHB mutations, a PASS score higher than 6, a high Ki-67 index are indexes for potential malignancy. Metastases can be present at first diagnosis or occur years after primary surgery. Measurement of plasma and/or urinary metanephrine, normetanephrine and metoxytyramine are recommended for biochemical diagnosis. Anatomical and functional imaging using different radionuclides are necessary for localization of tumor and metastases. Metastatic pheochromocytomas/paragangliomas is incurable. When possible, surgical debulking of primary tumor is recommended as well as surgical or radiosurgical removal of metastases. I-131-MIBG radiotherapy is the treatment of choice although results are limited. Chemotherapy is reserved to more advanced disease stages. Recent genetic studies have highlighted the main pathways involved in pheochromocytomas/paragangliomas pathogenesis thus suggesting the use of targeted therapy which, nevertheless, has still to be validated. Large cooperative studies on tissue specimens and clinical trials in large cohorts of patients are necessary to achieve better therapeutic tools and improve patient prognosis.

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“…In MRI these tumours have classically been described to have a "light-bulb" hyperintensity on T2-weighted sequences, due to a cystic component, and are hypointense on T1 sequences (14). The signal intensity on T2-weighted images may be low in malignant lesions due to haemorrhagic and necrotic areas though not a sufficient discriminating feature (15). The most specific radiotracer used is I 123 or I 131 labeled metaiodo benzyl guanidine (MIBG), which has chemical similarities to norepinephrine and is concentrated in chromaffin tissue.…”

Section: Malignant Phaeochromocytoma -A Challenge In Diagnosis and Thmentioning

confidence: 99%

Malignant phaeochromocytoma – a challenge in diagnosis and therapy

Dharshini

Bulugahapitiya²

2014

Sri Lanka J. Diabetes Endocrinol. Metab.

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Phaeochromocytoma is an endocrine tumour that originates in catecholamine producing chromaffin cells of the adrenal medulla. Approximately 10% are malignant but there are no precise histological or biochemical markers to distinguish these from benign ones. The presence of metastases at distant sites is the most reliable clue but histologic features utilized in several scoring systems aid in predicting malignancy. Malignant phaeohromocytoma predominantly secrete noradrenaline and there may be high dopamine levels. Increased levels of chromogranin A, negative staining for inhibin/activin beta subunit and presence of SDHB mutation are the other factors associated with malignant potential. Multi modality evaluation with combination of CT, MRI, SPECT and radionuclide scintygraphy augments the diagnostic yield. Recent advance in molecular diagnostic markers further improved the knowledge in predicting malignant potential. Currently available therapeutic options are surgical debulking, pharmacological therapy for excess catecholamines, radionuclide therapy, antineoplastic therapy and external radiotherapy. These modalities provide symptomatic relief and biochemical control, but with no significant survival benefit. The development in the field of molecular pathway responsible for the malignant potential of phaeochromocytoma gives a hope to future therapy.

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Incidental and Metastatic Adrenal Masses

Cited by 18 publications

References 81 publications

Subclinical phaeochromocytoma

Subclinical phaeochromocytoma

Updated and New Perspectives on Diagnosis, Prognosis, and Therapy of Malignant Pheochromocytoma/Paraganglioma

Malignant phaeochromocytoma – a challenge in diagnosis and therapy

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