Recognition and management of phaeochromocytoma and paraganglioma

Publication date: Available online 17 August 2017 Source:Anaesthesia & Intensive Care Medicine Author(s): Henry Wang, Chandran Jepegnanam Phaeochromocytomas and paragangliomas (PPGL) are catecholamine-secreting neuroendocrine tumours arising from the chromaffin cells in the adrenal medulla. These tumours may be identified incidentally, as part of a work-up for multiple endocrine neoplasia or following haemodynamic surges during unrelated procedures. Advances in perioperative management and improved management of intraoperative haemodynamic instability have significantly reduced surgical mortality from around 40% to less than 3%. Surgery is the definitive treatment in most cases and laparoscopic resection where possible is associated with improved outcomes. Anaesthetic management of PPGL cases represents a unique haemodynamic challenge both before and after tumour resection. In this article we describe the physiology of these tumours, their diagnosis, preoperative optimization methods, intraoperative anaesthetic management and management of postoperative complications.
Source: Anaesthesia and intensive care medicine - Category: Anesthesiology Source Type: research

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HPH Abstract Chromaffin tumors, e.g. pheochromocytomas and paragangliomas are caused by germline mutations of several susceptibility genes in 30-40% of the patients. The corresponding syndromes are multiple endocrine neoplasia type 2 (MEN2, RET gene), von Hippel-Lindau disease (VHL), neurofibromatosis type 1 (NF1), paraganglioma syndrome types 1-5 (PGL1-5, SDHx gene) and familial pheochromocytoma due to mutations in the MAX and TMEM127 genes. Clinically, screening for such diseases should be carried out by clinical symptoms and mutation analyses. Important indications can be found in the history of ...
Source: Der Chirurg - Category: Surgery Authors: Tags: Chirurg Source Type: research
VHL is another type of multiple endocrine neoplasia, and is inherited in an autosomal dominant manner like MEN1. Some families may have the VHL diagnosis without genetic testing, due to two or more clinical manifestations having been noted. The gene mutation is located at 3q26 –25, so the long arm of chromosome 3, locus 26–25. The most common tumours identified within this syndrome are retinal and central nervous haemangioblastomas, renal cancers, renal, pancreatic and epididymal cysts, phaeochromocytomas and paragangliomas (Maher, Neumann,&Richard, 2011).
Source: Journal of Pediatric Nursing - Category: Nursing Authors: Source Type: research
1335Objectives: :The aim of this study is to evaluate clinical role of 18F-DOPA PET/CT in neuroendocrine tumors (NET) and pheochromocytoma/paraganglioma (PHEO/PGL). Methods: Fifteen patients with diagnosed or clinically suspected NET and seventeen patients with suspected pheochomocytoma/paraganglioma were consecutively enrolled in this study. All patients undertook 18F-DOPA PET/CT. RET mutation was addressed in patients with suspected multiple endocrine neoplasia. Final diagnosis was confirmed by histopathology and clinical follow-up. Diagnostic efficacy was analyzed on per-patient and per-lesion basis respectively. Result...
Source: Journal of Nuclear Medicine - Category: Nuclear Medicine Authors: Tags: Endocrine/Neuroendocrine Posters Source Type: research
CONCLUSIONS: PET imaging with different radiotracers is a useful functional imaging technique in the work-up of several endocrine tumours. PMID: 28949122 [PubMed - as supplied by publisher]
Source: Minerva Endocrinologica - Category: Endocrinology Tags: Minerva Endocrinol Source Type: research
Václavíková E, Widimský J Abstract This work discusses the clinical performance of chromogranin A, free metanephrine and normetanephrine determination in plasma using a radioimmunoanalytical methods for the diagnosis of pheochromocytoma and paraganglioma. Blood samples were collected from 55 patients (46 pheochromocytomas, 9 paragangliomas). A sampling of biological materials was performed preoperatively and about one week, six months and one year after adrenal gland surgery. The comparative group without a diagnosis of pheochromocytoma/paraganglioma consisted of 36 pheochromocytoma/par...
Source: Physiological Research - Category: Physiology Authors: Tags: Physiol Res Source Type: research
We examined perioperative outcomes of pheochromocytoma/paraganglioma resection in 3 syndromic forms.
Source: Surgery - Category: Surgery Authors: Tags: Original Communication Source Type: research
Abstract: Hereditary tumor syndromes are increasingly recognized in the development of endocrine neoplasms. Depending on the tumor type, 10% to 40% of cases are associated with genetic disorders, including the classic multiple endocrine neoplasia syndromes (MEN1 and MEN2), hyperparathyroidism–jaw tumor syndrome, SDH-related familial paraganglioma-pheochromocytoma syndromes, von Hippel-Lindau syndrome, neurofibromatosis type 1, Carney complex, McCune-Albright syndrome, and familial nonmedullary thyroid cancer syndromes, as well as newer entities (MEN4, DICER1 syndrome, glucagon cell hyperplasia and neoplasia syndrome)...
Source: Pathology Case Reviews - Category: Pathology Tags: Reviews Source Type: research
ConclusionComposite pheochromocytoma/paraganglioma associated with multiple endocrine neoplasia type 2 is a very rare occurrence, as the current literature provides only a few cases. Further reported cases are needed in order to understand the behavior and the pathogenesis of this uncommon entity.
Source: Journal of Medical Case Reports - Category: General Medicine Source Type: research
Pheochromocytomas (PCC) and paragangliomas (PGL) are tumours occurring in the adrenal medulla and in extra-adrenal paraganglia, respectively. They have long been associated with familial occurrence and several syndromes had been described in which PCC formed an important component, including multiple endocrine neoplasia type 2, von Hippel-Lindau disease and neurofibromatosis type 1. Since the beginning of this millennium, both by the elucidation of specific genes, such as the various succinate dehydrogenase genes, as well as by generic molecular biology approaches, such as The Cancer Genome Atlas (TCGA) initiative, it was ...
Source: Diagnostic Histopathology - Category: Pathology Authors: Tags: Endocrine pathology Source Type: research
Conclusions: 68Ga-DOTATATA PET/CT could be the nuclear medicine imaging choice to evaluate suspected primary pheochromocytoma or paraganglioma, especially in the situation of multiple endocrine neoplasia syndrome.
Source: Clinical Nuclear Medicine - Category: Nuclear Medicine Tags: Original Articles Source Type: research
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