Endocrine Abstracts

I would like to present a case of malignant paraganglionoma which first presented as a mass in right clavicle in a 32-year-old female at Cork University Hospital. Her other medical background included Type 2 diabetes controlled on oral hypoglycemic agents and hypertension. On further imaging, she was found to have another mass in peri aortic region and biopsy confirmed the diagnosis and further verified by MIBG scan in March, 2005. Molecular analysis confirmed deletion of exon three of SDHB gene done at West Midlands Regional Genetic Laboratory, Birmingham. Patient underwent laparotomy and complete resection of the peri aortic mass in August, 2006. She also underwent radiation to the clavicular lesion with good response. MIBG and octreotide scans in December, 2005 were undertaken (MIBG only highlighted the clavicular lesion while octreotide was necessary for the para-aortic region. She later developed a pathological fracture of right Femur and underwent DHS insertion followed by radiotherapy to right hip. At that point, she was referred for targeted therapy to Kings College London under Dr Thomas. Pre-targeted therapy investigations revealed again disease in the right clavicle with the possibility of further in the sternoclavicular joint. Further disease in segment eight of the liver and multiple lesions in the lungs, lesions were also highlighted at the clavicular and right hip regions as well. Patient underwent four sessions of octreotide based chemotherapy between May and October, 2007 at Kings College Hospital, London. She also completed 5 sessions of radiotherapy to bony skeleton from September, 2005 to February, 2008. Catecholamines and dopamine levels were raised at diagnosis and pretargeted therapy (Nor adrenaline 19 348, urine normetadrenaline 51 328, urine three O methyl dopamine 14 256 and Nor adrenaline 6391,chromogranin 1694). She was readmitted with profuse vomiting in January, 2008 and investigations showed multifocal bony metastasis including cord compression. Ct showed progression of the lung metastasis however liver metastasis remained stable. At this stage, she was not deemed fit for radiotherapy or targeted therapy and palliative approach was followed. Patient was treated with high dose of alpha blockers, beta blockers, steroids and anti emetics to control the symptoms. Her conditioned continued to deteriorate and died in the 1st week of March, 2008.