A 63-year-old woman was admitted with a year's history of exertional breathlessness, anxiety attacks, syncopal episodes, diarrhoea, fatigue, reduced appetite, 2 stones weight loss, and flushing affecting her face and trunk. Investigations revealed raised urine 5-hydroxy indole acetic acid (5-HIAA) and chromogranin A. CT scan demonstrated extensive soft tissue encasing the major vessels intra-abdominally, and a retroperitoneal mass. (111)In-octreotide single-photon emission CT (SPECT CT) showed increased focal activity in the mediastinum, retroperitoneum and mesenteric lymph nodes. Para aortic lymph node biopsy confirmed the diagnosis of metastatic well-differentiated grade 1 gastrointestinal neuroendocrine tumour (NET). Extensive cardiac investigations confirmed severe mitral regurgitation, moderate aortic and tricuspid regurgitation, and mild pulmonary regurgitation. The patient's symptoms of flushing and diarrhoea were controlled with octreotide LAR, and she underwent mechanical aortic and mitral valve replacement. Following discharge from surgery, she went on to develop hydronephrosis and urosepsis, followed by infective endocarditis, resulting in recurrent admissions, and eventually passed away in her sleep nearly 14 months after her initial diagnosis.
2016 BMJ Publishing Group Ltd.