BACKGROUND: About 24 per cent of phaeochromocytomas (PCCs) and sympathetic paragangliomas (sPGLs) appear in familial cancer syndromes, including multiple endocrine neoplasia type 2, von Hippel-Lindau disease, neurofibromatosis type 1 and PCC-paraganglioma syndrome. Identification of these syndromes is of prime importance for patients and their relatives. Surgical resection is the treatment of choice for both PCC and sPGL, but controversy exists about the management of patients with bilateral or multiple tumours. METHODS: Relevant medical literature from PubMed, Ovid and Embase websites until 2009 was reviewed for articles on PCC, sPGL, hereditary syndromes and their treatment. DISCUSSION: Genetic testing for these syndromes should become routine clinical practice for those with PCC or sPGL. Patients should be referred to a clinical geneticist. Patients and family members with proven mutations should be entered into a standardized screening protocol. The preferred treatment of PCC and PGL is surgical resection; to avoid the lifelong consequences of bilateral adrenalectomy, cortex-sparing adrenalectomy is the treatment of choice. Copyright (c) 2009 British Journal of Surgery Society Ltd.
BACKGROUND: About 24 per cent of phaeochromocytomas (PCCs) and sympathetic paragangliomas (sPGLs) appear in familial cancer syndromes, including multiple endocrine neoplasia type 2, von Hippel-Lindau disease, neurofibromatosis type 1 and PCC-paraganglioma syndrome. Identification of these syndromes is of prime importance for patients and their relatives. Surgical resection is the treatment of choice for both PCC and sPGL, but controversy exists about the management of patients with bilateral or multiple tumours. METHODS: Relevant medical literature from PubMed, Ovid and Embase websites until 2009 was reviewed for articles on PCC, sPGL, hereditary syndromes and their treatment. DISCUSSION: Genetic testing for these syndromes should become routine clinical practice for those with PCC or sPGL. Patients should be referred to a clinical geneticist. Patients and family members with proven mutations should be entered into a standardized screening protocol. The preferred treatment of PCC and PGL is surgical resection; to avoid the lifelong consequences of bilateral adrenalectomy, cortex-sparing adrenalectomy is the treatment of choice. Copyright (c) 2009 British Journal of Surgery Society Ltd.
Authors: Pier Francesco Alesina; Jakob Hinrichs; Beate Meier; Kurt W Schmid; Hartmut P H Neumann; Martin K Walz Journal: Langenbecks Arch Surg Date: 2011-09-21 Impact factor: 3.445
Authors: Niamh X Cawley; William C Wetsel; Saravana R K Murthy; Joshua J Park; Karel Pacak; Y Peng Loh Journal: Endocr Rev Date: 2012-03-07 Impact factor: 19.871
Authors: David J Pinato; James R M Black; Ramya Ramaswami; Tricia M Tan; Delali Adjogatse; Rohini Sharma Journal: Med Oncol Date: 2016-04-08 Impact factor: 3.064
Authors: Susan A Elmore; Famke Aeffner; Dinesh S Bangari; Torrie A Crabbs; Stacey Fossey; Shayne C Gad; Wanda M Haschek; Jessica S Hoane; Kyathanahalli Janardhan; Ramesh C Kovi; Gail Pearse; Lyn M Wancket; Erin M Quist Journal: Toxicol Pathol Date: 2017-11-07 Impact factor: 1.902