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Malignant pheochromocytomas and paragangliomas: a diagnostic challenge
Linköping University, Department of Clinical and Experimental Medicine. Linköping University, Faculty of Health Sciences. Östergötlands Läns Landsting, Center for Surgery, Orthopaedics and Cancer Treatment, Department of Surgery in Östergötland.
Fac Med, Lab Anat and Cytologie Pathol, Marseille.
University of Bern.
University of Lyon 1.
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2012 (English)In: Langenbeck's archives of surgery (Print), ISSN 1435-2443, E-ISSN 1435-2451, Vol. 397, no 2, 155-177 p.Article, review/survey (Refereed) Published
Abstract [en]

Introduction Malignant pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare disorders arising from the adrenal gland, from the glomera along parasympathetic nerves or from paraganglia along the sympathetic trunk. According to the WHO classification, malignancy of PCCs and PGLs is defined by the presence of metastases at non-chromaffin sites distant from that of the primary tumor and not by local invasion. The overall prognosis of metastasized PCCs/PGLs is poor. Surgery offers currently the only change of cure. Preferably, the discrimination between malignant and benign PCCs/PGLs should be made preoperatively. less thanbrgreater than less thanbrgreater thanMethods This review summarizes our current knowledge on how benign and malignant tumors can be distinguished. less thanbrgreater than less thanbrgreater thanConclusion Due to the rarity of malignant PCCs/PGLs and the obvious difficulties in distinguishing benign and malignant PCCs/PGLs, any patient with a PCC/PGL should be treated in a specialized center where a multidisciplinary setting with specialized teams consisting of radiologists, endocrinologist, oncologists, pathologists and surgeons is available. This would also facilitate future studies to address the existing diagnostic and/or therapeutic obstacles.

Place, publisher, year, edition, pages
Springer Verlag (Germany) , 2012. Vol. 397, no 2, 155-177 p.
Keyword [en]
Pheochromocytoma, Paraganglioma, Malignancy, Diagnosis, Therapy
National Category
Medical and Health Sciences
URN: urn:nbn:se:liu:diva-75902DOI: 10.1007/s00423-011-0880-xISI: 000300493600003OAI: diva2:510547
Available from: 2012-03-16 Created: 2012-03-16 Last updated: 2014-11-03

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Gimm, Oliver
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Department of Clinical and Experimental MedicineFaculty of Health SciencesDepartment of Surgery in Östergötland
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