Endocrine Abstracts

Introduction: Histology is a poor predictor of the malignant potential of phaeochromocytomas and paragangliomas (PPGL) and only the presence of distant metastasis confirms malignancy. This study reviews patterns of recurrence, treatment and mortality in patients with malignant PPGL presenting to our department at UCLH over 30 years.Materials and Methods: In our series of 128 patients with PPGL, 26 (20%) with maligna...

Introduction: Histology is a poor predictor of the malignant potential of phaeochromocytomas and paragangliomas (PPGL) and only the presence of distant metastasis confirms malignancy. This study reviews patterns of recurrence, treatment and mortality in patients with malignant PPGL presenting to our department at UCLH over 30 years.Materials and Methods: In our series of 128 patients with PPGL, 26 (20%) with maligna...

Background: GEP-NETS and PPGLs in pediatric patients are rare; however, recognition of these diseases has increased recently. 10–20% of pediatric patients with GEP-NETs and up to 47% of pediatric patients with PPGLs present with metastatic disease at diagnosis. The disease is often unresectable with poor prognosis, and very few non-surgical therapies are approved for these patients. Due to paucity of data surrounding treatments for pediatric patients with advanced GEP-NET...

We present the case of a 53 year old gentleman with recurrent bladder paraganglioma. He presented initially in 2010 with headache and blurred vision when passing urine. The tumour was surgically removed, BP normalized and his postoperative MIBG-scan was normal. The patient presented with hypertensive crisis 4 years later with a suspicion of tumor recurrence, confirmed biochemically and radiologically. Treatment with phenoxybenzamine and bisoprolol resulted in adequate BP contr...

Background: Patients with Multiple Endocrine Neoplasia type 2 (MEN2) without previous family history often present late with advanced Medullary Thyroid Cancer (MTC). Surgery is not always curative but RET tyrosine kinase pathway is a potential target for molecular treatment for progressive MTC.Methods: Retrospective review of clinical, genetic, biochemical (calcitonin, CEA) and imaging (US, CT/MRI, Gallium Dotate) data of children with MEN2 who developed...