A combined glucagonoma and VIPoma syndrome: First pathologic and clinical report

Abstract

We report a case of pancreatic tumour metastatic to the liver in a patient with insulin-treated diabetes, anaemia, cheilitis, necrolytic migratory erythema, hypokalemia and chronic watery diarrhea, a picture suggesting combined glucagonoma and VIPoma syndromes. Immunocytochemistry of a biopsied hepatic metastatic nodule revealed both glucagon and vasoactive intestinal peptide (VIP) positive cells. Increased plasma glucagon and VIP levels were detected (values of 900 pmol/l and 277 pmol/l respectively). This is the first reported case showing not only immunocytochemical, but also clinical evidence of the combined secretion of these hormones.

Necrolytic migratory erythema: clinicopathologic study of 13 cases

“…Diagnostic tests demonstrate an increased erythrocyte sedimentation rate, hypoproteinemia, normochromic, normocytic anemia, abnormal glucose tolerance, increased glucagon levels, and decreased amino acid levels 16 . Rarely, clinical symptoms of gastrin‐secreting or vasoactive intestinal polypeptide‐secreting islet cell carcinoma (Zollinger–Ellison and Verner–Morrison syndromes) develop or precede NME 22 . In spite of these considerations, the symptoms are usually insidious and often begin 2 years before the diagnosis is made.…”

Multimodal Treatment of Vasoactive Intestinal Polypeptide-producing Pancreatic Neuroendocrine Tumors with Liver Metastases

“…During the 30-year period from 1985 to 2015, we identified 6 cases of VIPoma in the English literature (based on a PubMed search) that were managed by non-surgical multidisciplinary modalities alone (22-26). These six cases plus ours are summarized in Table 2.…”

“…Survival for at least 10 years was achieved in 3 cases with drugs or drugs plus TACE. Two patients died: one with severe hypokalaemia associated with watery diarrhoea (22) and one whose increasingly severe diarrhoea could no longer be controlled, despite the use of a somatostatin analogue and repeated TACE (24). The fact that exacerbation of symptoms was the cause of death in two cases and that survival of at least 10 years was seen in other cases as a result of a combination of chemotherapy and TACE suggests that control of endocrine symptoms in unresectable VIPoma is important to improve the prognosis.…”