SFEBES2016 ePoster Presentations (1) (116 abstracts)
Wrexham Maelor Hosital, Wrexham, UK.
A 52 years old man was referred to the renal clinic for persistent proteinurea and leg swelling with suspicion of nephrotic syndrome. He had a history of Hypertension and chronic depression and was taking olanzapine, venlafaxine, Ramipril.He also had diarrhoea of 56 stools per day over the last 6 months. He was getting short of breath and had lost weight. On examination he had erythematous rash over the face, limbs and abdomen which he mentioned was exacerbated by stress intermittently. He had a palpable liver of 34 cm and there was evidence of lower limb and peri-orbital swelling. The initial suspicion was that he has membranous glomerulonephritis associated with underlying malignancy. His abdominal US showed grossly enlarged and irregular liver containing multiple lesions of varying echotextures which were confirmed on CT along with a well defined lesion in the small bowel mesentry.Liver biopsy confirmed neuroendocrine tumour showing strong positive staining for CD56, chromogranin, NSE and synaptophysin. Ki67 shows 3% of the cells with positivity. Echo showed right sided heart involvement with severe tricuspid regurgitation and pulmonary stenosis. Repeat echo one week later showed new finding of aortic regurgitation suggesting left heart involvement.
24 hours Urinary 5-HIAA was significantly raised at 1384 micromole. A diagnosis of carcinoids syndrome was made.
Carcinoids are slow growing neuroendocrine tumours originating from enterochromaffin cells. Metastasis to the liver manifest as carcinoids syndrome via 5-HIAA and typically cause diarrhoea, flushing, wheeze and right sided cardiac involvement.
Apart from the typical features of carcinoids syndrome our case highlighted some unusual features like possible membranous GN and severe right sided cardiac involvement extending to the left side due to excessive amines load.