Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2022) 87 P11 | DOI: 10.1530/endoabs.87.P11

UKINETS2022 Poster Presentations (15 abstracts)

Metastatic VIPoma with Severe WDHA Syndrome: Natural History over 20 years documenting histological dedifferentiation - an illustrative case

Elspeth Saunders 1 , Bruce Sizer 2 , Jennifer Collins 1 , Srinivasan Gopalkrishnan 2 , Robert Skelly 1 , Robert Petty 1 & Mary McStay 1

1East Suffolk North Essex Foundation Trust, Colchester, United Kingdom; 2East Suffolk North Essex Foundation Trust, Ipswich, United Kingdom

A 51 year old female patient presented in 2000 with a 7 month history of diarrhoea and weight loss. Bloods revealed hypokalaemia (1.9). Infection and malabsorption screen negative, but patient remained hypokalaemic despite maximum iv and oral replacement. No abnormality was found endoscopically. Fasting gut hormone profile showed raised Vasoactive Intestinal Polypeptide (VIP) >400 (NR <30). CT scan confirmed 5cm mass in body of pancreas, no metastatic disease. Response to Octreotide was confirmed initially and histology confirmed VIPoma with Ki67 <5%. Symptoms returned with hypokalaemic myopathy 5 months later and after repeat octreotide scan again showed no metastases, distal pancreatectomy was performed. On surveillance, repeat imaging and gut hormones remained normal. However, over 10 years later in 2011, patient developed further diarrhoea with CT scan revealing recurrence in pancreatic bed. She was started on sandostatin analogues, and in 2012 had further resectional surgery of pancreatic bed disease (Ki67 5%). By June 2014, patient developed diarrhoea and although CT scans remained stable, chromogranins were raised. A gallium PET scan revealed multifocal serosal metastases. Patient was treated with PRRT, 4 cycles from June 2015 to May 2016 stabilizing disease. She was then retreated in 2018 but then presented with small bowel obstruction and was operated on at Specialist Peritoneal Malignancy unit. This revealed metastatic NET (Ki67 now 33%) and was advised to have adjuvant chemotherapy which patient declined. There was further evidence of progressive disease by 2021, and patient commenced FCarbo-Strep. Unfortunately, mid-treatment serial CT scan confirmed progression. Octreotide infusion 600 mg/24h was started and regular Lanreotide increased to 120mg every 2 weeks, but there was no relief from symptoms, developing hypokalaemia, hypomagnesaemia and hypotension. A trial of dexamethasone 4mg OD was prescribed. We increased octreotide infusion to 1.2mg/24h. Diarrhoea improved to a manageable level and electrolytes normalised. She was readmitted 8 weeks later with hypoglycaemia and progressively worsening diarrhoea despite ongoing octreotide infusion. Octreotide was titrated up to 1.5mg/24h and diarrhoea again settled. With limited further treatment she was referred for further PRRT. Unfortunately, patient deteriorated and was not fit enough for further treatment. She died December 2021, 21 years after diagnosis.

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