Abstract
Phaeochromocytoma is an endocrine tumour that originates in catecholamine producing chromaffin cells of the adrenal medulla. Approximately 10% are malignant but there are no precise histological or biochemical markers to distinguish these from benign ones. The presence of metastases at distant sites is the most reliable clue but histologic features utilized in several scoring systems aid in predicting malignancy. Malignant phaeohromocytoma predominantly secrete noradrenaline and there may be high dopamine levels. Increased levels of chromogranin A, negative staining for inhibin/activin beta subunit and presence of SDHB mutation are the other factors associated with malignant potential. Multi modality evaluation with combination of CT, MRI, SPECT and radionuclide scintygraphy augments the diagnostic yield. Recent advance in molecular diagnostic markers further improved the knowledge in predicting malignant potential. Currently available therapeutic options are surgical debulking, pharmacological therapy for excess catecholamines, radionuclide therapy, antineoplastic therapy and external radiotherapy. These modalities provide symptomatic relief and biochemical control, but with no significant survival benefit. The development in the field of molecular pathway responsible for the malignant potential of phaeochromocytoma gives a hope to future therapy. DOI: http://dx.doi.org/10.4038/sjdem.v4i1.7253 Sri Lanka Journal of Diabetes, Endocrinology and Metabolism 2014; 4 : 40-42
Highlights
Catecholamine secreting tumours are rare neoplasms that arise from chromaffin cells of the adrenal medulla and the sympathetic ganglia
And biochemically malignant tumours cannot be differentiated from benign ones
The presence of tumour spread to distant sites where chromaffin tissue is normally absent such as lymph nodes, liver, lung and bones is the only reliable clue to the presence of malignancy [4]
Summary
Catecholamine secreting tumours are rare neoplasms that arise from chromaffin cells of the adrenal medulla (phaeochromocytoma) and the sympathetic ganglia (paraganglioma). Around 10% of these tumours are malignant [2,3] and despite advanced diagnostic methods prediction of malignancy is difficult. And biochemically malignant tumours cannot be differentiated from benign ones. The presence of tumour spread to distant sites where chromaffin tissue is normally absent such as lymph nodes, liver, lung and bones is the only reliable clue to the presence of malignancy [4]. Standard therapies for malignant phaeochromocytoma are non specific and the rarity of this condition makes it difficult to gather knowledge regarding outcomes of new forms of therapy. Since malignant phaeochromocyoma carries a poor prognosis and the occurrence of metastasis even years after the primary surgery, improvement in diagnostic and therapeutic measures are crucial [5,6]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callMore From: Sri Lanka Journal of Diabetes Endocrinology and Metabolism
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
