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A Unique Apudoma Producing the Glucagonoma and Gastrinoma Syndromes J. DAWSON*T SR Bloomt R. COCKEL

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A Unique Apudoma Producing the Glucagonoma and Gastrinoma Syndromes J. DAWSON*T SR Bloomt R. COCKEL Postgrad Med J: first published as 10.1136/pgmj.59.691.315 on 1 May 1983. Downloaded from Postgraduate Medical Journal (May 1983) 59, 315-316 A unique apudoma producing the glucagonoma and gastrinoma syndromes J. DAWSON*t S. R. BLOOMt D.M., M.R.C.P. M.A., D.Sc., M.D., F.R.C.P. R. COCKEL* M.A., M.B., F.R.C.P. *Division of Medicine, Selly Oak Hospital, Raddlebarn Road, Birmingham B29 6JD and : Department of Medicine, Royal Postgraduate Medical School, Ducane Road, London W12 OHS Summary meal had suggested duodenal ulceration. Glycosuria had been noted on routine urinalysis in 1971. In 1977, A patient is described who presented with a 10 year he was admitted to a local skin hospital for investiga- history of intermittent peptic ulcer symptoms and a 3 tion of a severe generalised erythematous rash with year history of an undiagnosed skin rash. Investiga- patchy bullous changes, particularly severe on the tions indicated raised plasma levels of gastrin, lower abdomen, thighs and buttocks. No firm diag- glucagon and pancreatic polypeptide. A single tumour nosis was made of this rash which was variously was localised to the pancreas and resected. The described as atypical bullous pemphigoid or infected tumour had the typical histology of an apudoma, and discoid eczema. The rash eventually responded to copyright. contained cells which stained for gastrin, glucagon, oral tetracycline and fluorinated topical steroids. At pancreatic polypeptide and neurotensin. the time of the current admission, the patient was cachectic, anaemic and had a patchy macular rash KEY WORDS: apudoma, gastrointestinal hormones, gastrin, glucagon, with areas of confluence on trunk and legs. Abdomi- Zollinger-Ellison syndrome. nal palpation revealed an ill-defined epigastric Introduction mass. Blood tests showed a normocytic, normochromic It is becoming increasingly recognised that pan- anaemia (haemoglobin 9.1 g/dl), a raised erythrocyte http://pmj.bmj.com/ creatic endocrine tumours (apudomas) may contain sedimentation rate (50 mm/hr), elevated blood glu- more than one peptide hormone (Abe et al., 1979; cose (10 mmol/litre) and alkaline phosphatase (106.5 Heitz et al., 1979). Nevertheless, individual patients and a low serum albumin (25 g/litre). whose tumours have not metastasised present with a u./litre) single clinical syndrome due to over-production of Barium meal and upper gastrointestinal endoscopy one of the peptides. We report a patient who showed an expanded duodenal loop with multiple presented with the combined features of Zollinger- peptic ulcers extending throughout the duodenum. Ellison (gastrinoma) and glucagonoma syndromes in Gastric secretion tests showed a raised basal acid on September 27, 2021 by guest. Protected whom hypersecretion of 3 peptides was demon- concentration (83.8 mmol/litre; normal less than 40), but a normal basal acid output (3.8 mmol/hr; normal strated. less than 5). Fasting plasma gastrointestinal hor- mones showed a raised gastrin (306 pmol/litre, Case report normal less than 30 pmol/litre), raised glucagon (475 A 70-year-old man was admitted to hospital in pmol/litre, normal less than 50) and raised pan- May 1980 with a 3 month history of constant upper creatic polypeptide (1850 pmol/litre, normal less abdominal pain with associated nausea, anorexia, than 200) which failed to suppress with atropine weight loss (10 kg) and episodic diarrhoea. The (Bloom, Adrian and Polak, 1980). Somatostatin and symptoms had been partially relieved by cimetidine vasoactive intestinal peptide levels were normal. prescribed empirically. In 1971, he had been investi- Upper abdominal ultrasound, computed tomography gated for similar upper abdominal pain and a barium and arteriography localised a single avascular tu- mour in the head ofthe pancreas. The ulcers failed to t Present address: Department of Medicine, Queen Elizabeth respond to high-dose cimetidine therapy and, despite Hospital, Birmingham. continued therapy, his skin problems remained. He 0032-5473/83/0500-0315 $02.00 © 1983 The Fellowship of Postgraduate Medicine Postgrad Med J: first published as 10.1136/pgmj.59.691.315 on 1 May 1983. Downloaded from 316 Clinical reports therefore underwent laparotomy and a single 9 cm syndromes ascribed to hypergastrinaemia and hyper- diameter hypovascular tumour was .removed from glucagonaemia in a single patient. This patient the head of the pancreas in a Whipple's procedure. undoubtedly had features ascribed to over-pro- Histological examination showed a typical endocrine duction of both hormones for 9 years, having been tumour which, on immunocytochemistry, was shown noted to have glycosuria and a duodenal ulcer in to contain approximately equal proportions of 3 1971 and severe clinical problems for 3 years before types of cells, staining for gastrin, glucagon, and diagnosis. The case emphasises the slow growth and pancreatic polypeptide respectively with, in addition, potential of endocrine pancreatic tumours to secrete a few sparsely scattered neurotensin cells. Electron more than one hormone. It demonstrates the need for microscopy showed the presence of 4 cell types in the awareness of the presence of such tumours when tumour. Radioimmunoassay of tumour extracts con- patients manifest unusual combinations of symp- firmed presence of gastrin (280 pmol/g wet weight) toms, even when these symptoms are of long stand- glucagon (119 pmol/g) and pancreatic polypeptide ing. (80 pmol/g). Postoperative measurements of fasting plasma hormones showed that these had fallen to Acknowledgments normal levels indicating origin from the single We thank Dr J. M. Polak for performing the histochemical tumour. The patient, following an initial good studies, and Mrs Dawn Campbell for typing the manuscript. recovery, died after recurrent surgery for persistent pancreatic anastomosis fistula problems 6 weeks after References surgery. ABE, K., YAMAGUCHI, K., ADACHI, I. & YANAIHARA, N. (1979) Multiple hormone production in islet cell tumours. In: Proinsulin, Discussion Insulin and C-peptide (Eds. S. Baba, T. Kaneho and N. Yanihara), p. 374. Excerpta Medica, Amsterdam. Pancreatic polypeptide production has been de- BLOOM, S.R., ADRIAN, T.E. & POLAK, J.M. (1980) Pancreatic scribed previously in patients with pancreatic endo- polypeptide from pancreatic endocrine tumours. Lancet, ii, 1026. crine tumours associated with typical syndromes and BRODER, L.E. & CARTER, S.K. (1973) Pancreatic islet cell carci- does not, in noma. I. clinical features of 52 patients II. results of therapy withcopyright. itself, produce symptoms (Welbourn et streptomycin in 52 patients. Annals of Internal Medicine, 79, 101. al., 1981). However, although secretion ofgastrin and HEITZ, P.U., KASPER, M., POLAK, J.M. & KLOPPEL, G. (1979) glucagon has been reported in a patient who pre- Pathology of the endocrine pancreas. Journal of Histochemistry sented with an insulinoma (Broder and Carter, 1973), and Cytochemistry, 27, 1401. no WELBOURN, R.B., WOOD, S.M., POLAK, J.M. & BLOOM, S.R. (1981) there have been previous reports of the hyperse- Pancreatic endocrine tumours. In: Gut Hormones (Eds. S. R. cretion of gastrin, glucagon and pancreatic polypep- Bloom and J. M. Polak), 2nd edn., p. 547. London, Churchill tide and no reports of the co-existence of the clinical Livingstone, Edinburgh and London. http://pmj.bmj.com/ (Accepted 20 October 1982) on September 27, 2021 by guest. Protected.
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