Phaeochromocytoma and paraganglioma.

[Phaeochromocytoma and paraganglioma]. Rev Med Interne. 2019 Sep 04;: Authors: Cornu E, Belmihoub I, Burnichon N, Grataloup C, Zinzindohoué F, Baron S, Billaud E, Azizi M, Gimenez-Roqueplo AP, Amar L Abstract Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors that arise from the adrenal medulla or sympathetic and parasympathetic ganglia. These tumors produce most often catecholamines in excess, causing hypertension and sometimes severe acute cardiovascular complications. The diagnosis is based on plasma or urines metanephrines measurements and on conventional and nuclear medicine imaging. Catecholamines-producing PPGL is very unlikely if levels are normal. The diagnosis of PPGL cannot be made without visualization of a tumor. Therapeutic management consists mostly of surgical excision, after drug preparation, and should be done in referral centers. About 40% of pheochromocytomas and paragangliomas occur in the context of an autosomal inherited syndrome, making genetic testing essential. The follow-up must be prolonged because a metastatic evolution or a recurrence can be observed in about 15% of the cases. PMID: 31493938 [PubMed - as supplied by publisher]
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research

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Karel Pacak Pheochromocytomas and paragangliomas (PPGLs) are rare catecholamine-secreting neuroendocrine tumors of the adrenal medulla and sympathetic/parasympathetic ganglion cells, respectively. Excessive release of catecholamines leads to episodic symptoms and signs of PPGL, which include hypertension, headache, palpitations, and diaphoresis. Intraoperatively, large amounts of catecholamines are released into the bloodstream through handling and manipulation of the tumor(s). In contrast, there could also be an abrupt decline in catecholamine levels after tumor resection. Because of such binary manifestations of PP...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Review Source Type: research
Conclusions: Preliminary analysis suggests that there is an increase in HR and SBP after 60 minutes of PHE/TYR-containing amino acid administration which, in patients with PHEO/PGL, may represent increased sympathetic effects from temporarily boosted catecholamine biosynthesis. This is supported by the finding that increased HR and SPB is seen primarily in catecholamine secretors when using Clinisol but not with the 2.5% Lys/Arg solution. Other factors which may have contributed such as anxiety and nausea cannot be ruled out, although subgroup analysis suggests that these others factors alone does not account for the obser...
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Center for Therapy Excellence YIA Symposium Source Type: research
Conclusions: 68Ga-DOTA-NOC PET/CT shows higher sensitivity in the detection of bone metastasis and lymphadenopathy in PHEOs/PGLs, which has great merits for staging and risk stratification of PHEOs/PGLs. PRRT has good response especially in symptom release and improvement of living quality, and is tolerable with no evident hematological events and renal toxicity. Furthermore, present study will pave the way for SSA based theranostics on PHEOs/PGLs. Keywords: Pheochromocytomas, Paragangliomas, 68Ga-DOTA-NOC,131I-MIBG, Somatostatin Receptor Abbreviations: PHEO: Pheochromocytoma; PGL: Paraganglioma; MIBG: Metaiodobenzylguanid...
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Endocrinology (Clinical Therapy) Posters Source Type: research
Abstract Pheochromocytomas and sympathetic paragangliomas are rare catecholamine-secreting tumors and represent very rare causes of intracerebral hemorrhage in young, with only few cases reported. A 32-year-old man presented to our emergency department because of sudden onset of severe headache. He had a six months history of paroxysmal headache, palpitations and sweating. During examination he became somnolent and developed left-sided hemiplegia. A computed tomographic (CT) scan of the brain showed a right temporoparietal hematoma. He was admitted to the Clinic for Neurosurgery and hematoma was evacuated. The pat...
Source: Endokrynologia Polska - Category: Endocrinology Authors: Tags: Endokrynol Pol Source Type: research
Postmicturition syndrome refers to symptoms caused by overdistension of the bladder or micturition. Bladder paraganglioma is a rare neuroendocrine neoplasm, which arises from the chromaffin tissue of the sympathetic nervous system embedded in the muscle layer of the bladder wall. Clinical presentation of catecholamine-secreting paragangliomas may mimic that of hyperfunctioning adrenal pheochromocytoma. Typical symptoms such as sweating, palpitations, headache, nausea, hypertension, or flushing are due to catecholamine release and are related to micturition or bladder overdistension.
Source: Journal of the American Society of Hypertension - Category: Cardiology Authors: Tags: Clinical Case Presentation Source Type: research
Post-micturition syndrome refers to symptoms caused by over distension of the bladder or micturition. Bladder paraganglioma (BP) is a rare neuroendocrine neoplasm, which arises from the chromaffin tissue of the sympathetic nervous system embedded in the muscle layer of the bladder wall. Clinical presentation of catecholamine-secreting paragangliomas may mimic that of hyperfunctioning adrenal phaeochromocytoma. Typical symptoms such as sweating, palpitations, headache, nausea, hypertension or flushing are due to catecholamine release and are related to micturition or bladder over-distension.
Source: Journal of the American Society of Hypertension - Category: Cardiology Authors: Tags: Clinical Case Presentation Source Type: research
Conclusions: LMI1195, a novel F-18-labeled PET-tracer, is highly effective in detecting tumors of the adrenal medulla or the sympathetic trunk. High uptake in lesions and superb image quality promote its use as alternative to MIBG. Further potential applications (e.g. assessment of the sympathetic innervation of the heart) should be evaluated.
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: First-in-Human & amp; New Human Applications Source Type: research
Conclusions: The mainstay of treatment for patients with PCC/PGLs is surgical. However, the tide may be turning with the discovery of new genes associated with PCC/PGLs that may shed light on oncometabolites utilized by these tumors. PMID: 29409060 [PubMed - as supplied by publisher]
Source: The Journal of Clinical Endocrinology and Metabolism - Category: Endocrinology Authors: Tags: J Clin Endocrinol Metab Source Type: research
Tumors of the sympathetic nervous system, both pheochromocytoma and paraganglioma release norepinephrine. While there is no catecholamine (norepinephrine; epinephrine) secretion in some paraganglioma cases, epinephrine is secreted in addition to norepinephrine in some pheochromocytoma cases [1]. Excessive norepinephrine leads to high blood pressure and excessive epinephrine to tachyarrhythmia [2]. Metanephrine and normetanephrine measurements are conducted for the biochemical diagnosis of pheochromocytoma and paraganglioma.
Source: Medical Hypotheses - Category: Biomedical Science Authors: Source Type: research
CONCLUSIONS: The measurement of plasma free MN and NMN with LC-MS/MS is not affected by use of β-blockers, diuretics and ACE inhibitors. Withdrawal of these drugs prior to the quantification of plasma metanephrines is therefore not necessary. PMID: 27291837 [PubMed - as supplied by publisher]
Source: Clinical Biochemistry - Category: Biochemistry Authors: Tags: Clin Biochem Source Type: research
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