Dopamine-secreting phaeochromocytomas and paragangliomas: clinical features and management
Document Type
Article
Publication Date
5-1-2010
Abstract
Most functional phaeochromocytomas/paragangliomas produce noradrenaline and/or adrenaline. Those that produce dopamine are rare. We describe the distinguishing clinical features of dopamine-secreting phaeochromocytomas and paragangliomas from those that secrete noradrenaline/adrenaline and the impact on their management. We present a case of a dopamine-secreting paraganglioma from our institution and review 14 case reports of dopamine-secreting phaeochromocytomas/paragangliomas published between 1984 and 2008. As observed in the literature, 80% of the tumours were extra-adrenal. Most patients presented with non-specific symptoms or mass effect without the classical presentation of catecholamine excess. The majority were diagnosed with urinary or plasma dopamine. Five patients had malignant tumours and 12 patients underwent surgical resection of the primary tumours. Unlike noradrenaline/adrenaline-secreting phaeochromocytomas/paragangliomas, dopamine-secreting tumours lack a classical presentation, are extra-adrenal and have a higher malignant potential. A routine inclusion of urinary or plasma dopamine as part of catecholamine screening in all suspected phaeochromocytomas and paragangliomas is recommended.
Keywords
Abdominal Neoplasms/diagnosis, Abdominal Neoplasms/pathology
Divisions
fac_med
Publication Title
Singapore Medical Journal
Volume
51
Issue
5
Publisher
Stamford Publishing Pte Ltd / Singapore Medical Association
Additional Information
Endocrine Unit, Department of Medicine, University Malaya Medical Centre, Lembah Pantai, Kuala Lumpur 59100, Malaysia. jsiewhui@yahoo.com