PubMed · 6294887
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J Chleboun. 1982. Vipoma.. https://pubmed.ncbi.nlm.nih.gov/6294887/
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Congenital hyperinsulinism (HI) exists in 2 histologic forms, focal and diffuse, and rarely has been attributed to lesions in ectopic pancreatic tissue. The ability to distinguish focal from diffuse HI and locate focal lesions has been difficult, thus limiting the optimal management of HI. We present a case of HI resulting from focal pancreatic and ectopic pancreatic lesions. After a near-total pancreatectomy failed to improve the patient's condition, a positron emission tomography (PET) scan performed with 18F-fluoro-L-dihydroxyphenylalanine demonstrated a focal lesion remaining in the head of the pancreas as well as 4 hot spots inferior to the remaining pancreas. Surgical exploration found pancreatic rests in the jejunum responsible for the hot spots seen on PET. Resection of the remainder of the pancreas as well as the small intestinal lesions resulted in correction of the patient's HI. Pathology confirmed the presence of focal HI lesions in the pancreatic head and small intestinal specimens. This case supports the ability of ectopic pancreatic tissue to contribute to the pathology of HI. It highlights the ability of PET to successfully identify focal lesions, including ectopic tissue, responsible for hyperinsulinemic hypoglycemia.
OBJECTIVE: We and others have reported loss of heterozygosity (LOH) on chromosome 3p25 in sporadic malignant pancreatic endocrine tumours (PETs). A common region of deletion on chromosome 3p25 contains numerous genes, including VHL and PPARgamma, that have been excluded previously as candidate tumour suppressor genes by DNA sequencing analysis. We have analysed whether WNT7A or HDAC11 was biallelically inactivated in a group of well-characterized PETs. PATIENTS AND DESIGN: Ten PETs from eight patients were selected from a previous study, where LOH on chromosome 3p25 was found in 11 out of 22 sporadic PETs. These tumours were examined for inactivating mutations of WNT7A and HDAC11 by direct sequencing of all exons and intron-exon boundaries. Inactivation of WNT7A expression by aberrant CpG island methylation and WNT7A protein expression were evaluated by methylation-specific polymerase chain reaction (PCR) and immunohistochemistry, respectively. HDAC11 protein expression was also examined. RESULTS: No point mutations, deletion or insertions were detected in either WNT7A or HDAC11 in any of the PETs. Two polymorphisms were identified in the third exon of the WNT7A gene. CpG methylation of the WNT7A gene was not detected and the WNT7A and HDAC11 proteins were normally expressed. CONCLUSION: The absence of tumour-specific somatic events in WNT7A and HDAC11 suggests that these genes are unlikely to have a classical tumour suppressor gene role in sporadic malignant PETs. The putative 3p25 tumour suppressor remains to be identified.
OBJECTIVE: Expression of ghrelin has been reported in pancreatic endocrine tumours, but data on ghrelin receptor protein expression are lacking. The aim of this study was to examine the ghrelin receptor, as well as ghrelin, in a selected series of these tumours, including multiple endocrine neoplasia 1 (MEN1) associated tumours, and to correlate data with clinical features including body mass index. DESIGN: Immunohistochemical detection of ghrelin and its receptor was performed on frozen tissue from 31 tumours: 9 MEN1 and 22 sporadic. Twenty tumours were analysed by quantitative PCR. Plasma ghrelin was assessed in 26 patients. RESULTS: Twenty-one (68%) of 31 tumours showed immunoreactivity for ghrelin (8/9 MEN1) and 19/20 expressed ghrelin mRNA. Ghrelin receptor protein was detected in 21/30 (70%) tumours (4/8 MEN1), and mRNA was detected in all analysed tumours. Insulinomas had significantly higher levels of receptor mRNA than other tumours. Five patients had elevated plasma ghrelin (> 2 SD above the control group mean). No significant difference in mean plasma ghrelin levels was found between patients (908 +/- 569 ng/l) and controls (952 +/- 164 ng/l). Mean BMI was 24.3 kg/m(2). There was no association between ghrelin or receptor expression and survival. CONCLUSIONS: We report the first immunohistochemical data on expression of the ghrelin receptor in pancreatic endocrine tumours: 70% of tumours in our material. Concomitant ghrelin and receptor expression was seen in 50% of tumours, indicating an autocrine loop. Ghrelin was expressed in 68% of tumours (8/9 MEN1). Despite frequent ghrelin expression, elevated circulating ghrelin is rare in these patients.