Familial pheochromocytoma

Zoran Erlic, Hartmut P.H. Neumann

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Pheochromocytomas and Paragangliomas (PGL) form the group of paraganglial tumours which can occur in any paraganglia from the skull base to the pelvic floor. The terminology is not uniform. While the World Health Organization (WHO) applies pheochromocytoma exclusively to adrenal tumours, many clinicians use the term pheochromocytoma also for extra-adrenal abdominal and thoracic tumours, since by tradition …

Diagnosis of pheochromocytoma with special emphasis on MEN2 syndrome

Karel Pacak, Graeme Eisenhofer, Ioannis Ilias

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Pheochromocytomas/paragangliomas(PHEOs/PGLs) are rare but treacherous catecholamine-producing tumors which, if overlooked or improperly treated, will almost invariably prove fatal. Patients with MEN2 PHEOs have a high incidence of paroxysmal attacks …

A novel succinate dehydrogenase type B mutation in an Iranian family. Its genetic and clinical evaluation

Ali A. Ghazi, Ali Mosaddegh Khah, Fereshteh Kamani, Khandan Zare, Alireza Sadeghipour, Mehdi Hedayati, Marjan Zarif Yeganeh, Treena Cranston, Ashley Grossman

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Succinate Dehydrogenase-B (SDH-B) gene mutations constitute one of the most frequent forms of hereditary paragangliomas (PGL). Genetic study is advised in all cases for the evaluation of tumour behaviour, the selection of optimal management and the surveillance of the first degree relative …

Familial paraganglioma: A novel presentation of a case and response to therapy with radiolabelled MIBG

Justin K. Lawrence, Eamonn R. Maher, Richard Sheaves, Ashley B. Grossman

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Phaeochromocytomas (PC) and paragangliomas are disorders of the sympatho-adrenomedullary system. They are chromaffin-containing neuroendocrine tumors of neural crest origin that contain catecholamine-secreting granules: they arise from either the adrenal medulla (phaeochromocytomas) or from extra-adrenal neural crest derivatives e.g. the sympathetic chain (paragangliomas)…

Clinical and biochemical responses after Gamma Knife surgery for a dopamine-secreting paraganglioma: case report

Constantin Tuleasca, Yves Jaquet, Valerie Schweizer, Laura Negretti, Vera Magaddino, Philippe Maeder, Karim-Alexandre Abid, Benoit Lhermitte, Eric Grouzmann, Marc Levivier

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INTRODUCTION: The efficacy of Gamma Knife surgery (GKS) in local tumor control of non-secreting paragangliomas (PGLs) has been fully described by previous studies. However, with regard to secreting PGL, only one previous case report exists advocating its efficacy at a biological level. CASE REPORT: The aims of this study were: 1) to evaluate the safety/efficacy of GKS in a dopamine-secreting …