Pheochromocytoma and paraganglioma : Importance of diagnostic imaging.

[Pheochromocytoma and paraganglioma : Importance of diagnostic imaging]. Radiologe. 2019 Jul 23;: Authors: Kunz WG, Auernhammer CJ, Nölting S, Pfluger T, Ricke J, Cyran CC Abstract CLINICAL BACKGROUND: If pheochromocytoma (PC) or paraganglioma (PGL) is diagnosed based on serologic studies, imaging is required to locate the adrenal mass for further management. Besides pathognomonic hormonal findings, PC/PGL can exhibit typical imaging features. However, PC/PGL can also show morphological overlap with other pathologies. STANDARD RADIOLOGICAL METHODS: The modality of choice for evaluation of PC is CT. In case of extra-adrenal location, MRI is superior to CT. Imaging with PET-CT provides complementary information in the differentiation of PC/PGL and is recommended as the imaging modality of choice for malignant PC/PGL. 68Ga-DOTATATE (or 68Ga-DOTATOC/ 68Ga-DOTANOC) PET-CT has high sensitivity for SDHx-mutated PC/PGL and serves for planning of radioreceptor therapy with somatostatin analogues. In contrast, 123I-metaiodobenzylguanidine (MIBG) scintigraphy is important in assessing the potential efficacy of radioreceptor therapy with MIBG. METHODICAL DETAILS: The CT protocol for PC evaluation should include non-enhanced, arterial, portal-venous and late phases; the latter for the evaluation of wash-out. Recent studies indicate non-enhanced CT alone may be sufficient to rule out PC. For MRI, in- and opposed-phase sequences should be additional...
Source: Der Radiologe - Category: Radiology Authors: Tags: Radiologe Source Type: research

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To the Editor As academic physicians with an interest in pheochromocytoma/paraganglioma, we read with great interest the article by McCrary et al characterizing malignant head and neck paragangliomas (HNPGLs). The authors used the paraganglioma cohort from an academic tertiary cancer center diagnosed between 1963 and 2018. They found that the prevalence of malignant HNPGLs was 6 of 70 (9%), with 5 of 6 patients carrying the succinate dehydrogenase subunit B (SDHB) mutation. They suggested that patients with paragangliomas should undergo genetic testing, and owing to the difficulty in diagnosing malignant HNPGL prior to sur...
Source: JAMA Otolaryngology - Head and Neck Surgery - Category: ENT & OMF Source Type: research
AbstractParagangliomas are rare neuroendocrine tumors which originate from embryonic neural crest cells. These tumors may arise from parasympathetic or sympathetic paraganglia, may secrete catecholamines, and can occur in varied anatomic locations, with some locations being less common than others. Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas and pheochromocytomas and have been associated with germline heterozygous mutations inMAX,SDHA,SDHAF2,SDHB,SDHC,SDHD, orTMEM127. Herein, we report a case of a middle-aged male who was diagnosed with an intrarenal/renal pelvis paraga...
Source: Endocrine Pathology - Category: Pathology Source Type: research
New England Journal of Medicine,Volume 381, Issue 19, Page 1882-1883, November 2019.
Source: New England Journal of Medicine - Category: Internal Medicine Source Type: research
Conclusion: About 37% of PPGL without family history of such tumors harbor germline mutations. The most commonly mutated gene is SDHB followed by SDHC, SDHD, VHL, MAX and rarely RET, SDHA, SDHAF2, TMEM127 and NF1. SDHB mutations were associated with metastatic PPGL in more than a quarter of cases. PMID: 31666924 [PubMed]
Source: Oncotarget - Category: Cancer & Oncology Tags: Oncotarget Source Type: research
CONCLUSION: Our findings extend the spectrum of transcriptional dysregulations in PPGL to lncRNAs and provide a novel biomarker that could be useful to identify potentially metastatic tumors in patients carrying SDHx mutations. PMID: 31678991 [PubMed - as supplied by publisher]
Source: The Journal of Clinical Endocrinology and Metabolism - Category: Endocrinology Authors: Tags: J Clin Endocrinol Metab Source Type: research
Publication date: Available online 24 October 2019Source: Best Practice &Research Clinical Endocrinology &MetabolismAuthor(s): Sina Jasim, Camilo JimenezAbstractMetastatic pheochromocytomas and paragangliomas (MPPGs) are rare neuroendocrine tumors. Most patients present with advanced disease that is associated with manifestations of catecholamine release. Surgical resection of the primary tumor and ablative therapies of metastases—whenever possible—may improve clinical outcomes and, perhaps, lengthen the patient’s overall survival. Significant steps in understanding the genetic alterations linked ...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - Category: Endocrinology Source Type: research
This article summarizes current clinical approaches in patients with pheochromocytoma/paraganglioma.
Source: Endocrinology and Metabolism Clinics of North America - Category: Endocrinology Authors: Source Type: research
Publication date: Available online 22 October 2019Source: Best Practice &Research Clinical Endocrinology &MetabolismAuthor(s): Emilia Sbardella, Ashley B. GrossmanAbstractPheochromocytomas are rare neuroendocrine chromaffin-derived tumors that arise within the adrenal medulla. They are usually benign, but if not diagnosed or if left untreated, they can have devastating consequences. Clinical consideration of the diagnosis is paramount, as they may have protean manifestations, and a high index of suspicion is essential if serious consequences are to be avoided.An accurate biochemical diagnosis is crucial for the man...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - Category: Endocrinology Source Type: research
Publication date: Available online 21 October 2019Source: Best Practice &Research Clinical Endocrinology &MetabolismAuthor(s): Florentine Schreiner, Felix BeuschleinAbstractPheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors, a large proportion of which secrete catecholamines. PPGL are associated with a high cardiovascular morbidity and come with a risk of malignancy. The therapy of choice is surgical resection. Nevertheless, PPGL are associated with a lifelong risk of tumor persistence or recurrence. Currently, there are no clinical, biochemical, histopathological or imaging characteristics, w...
Source: Best Practice and Research Clinical Endocrinology and Metabolism - Category: Endocrinology Source Type: research
Conclusion: Patients chronically treated with lithium need to be screened for hypothyroidism, goiter, and hyperparathyroidism, as the prevalence of these endocrine abnormalities is higher in lithium-treated patients than in the general population. The growth inhibitory effects of lithium in medullary TC, pheochromocytoma/paraganglioma and carcinoid were achieved with supratherapeutic concentrations of lithium thus limiting its translational perspective. Currently available clinical data on the efficacy of lithium in the therapy of endocrine tumors in human is limited and associated with conflicting results.
Source: Frontiers in Oncology - Category: Cancer & Oncology Source Type: research
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