Endocrine Abstracts

A 52 year old man presented with a 4 week history of heartburn and epigastric pain. On examination 6 centimetres of irregular heptomegaly were palpable extending across to the epigastrium. Ultrasound of the abdomen demonstrated multiple liver metastases. Percutaneous liver biopsy was consistent with a neuroendocrine tumour (NET) with positive staining for CAM 5.2, chromogranin, PGP 9.5 and S100 but negative for alpha fetoprotein and CEA. Gamma glutamyl transpepitdase was elevated at 335 micromoles per litre but the transaminases were normal. Antigastric-parietal cell antibodies were negative. Fasting serum gastrin, pancreastatin, somatostatin and 24 hour urinary 5 hydroxytryptophan (5HT) were elevated but 24h urinary 5-hydroxyindoleacetic acid (5HIAA)was initially normal. The serum alpha fetoprotein was elevated at 290 kilo units per litre (normal range less than 10). A small bowel series and abdominal CT failed to localise the primary tumour. Octreotide scanning showed multiple foci of increased uptake consistent with liver metastases and a possible small metastases at the left lung base. Cardiac echocardiography was normal. He was commenced on omeprazole and monthly sandostatin injections and subsequently received 4 cycles of 3GBq yttrium 90 labelled octreotide with good symptomatic and hormonal response but no radiological change at 12 months after diagnosis. At this point his serum gastrin, pancreastatin and somatostatin levels fell significantly. The serum alpha fetoprotein also fell to 133 kilo units per litre. Over the next 8 months his clinical condition deteriorated. This coincided with an unchanged serum gastrin but a secondary rise in AFP and massively elevated 24h urinary 5HIAA and 5HT. The patient died 20 months after initial diagnosis.
An elevated AFP in NET patients is extremely rare and may be associated with a worse prognosis. Of note also is the changing pattern of hormonal secretion with time, presumptively associated with tumour de-differentiation.