Pancreatic Islet Cell Tumour in a Patient with Familial Adenomatous Polyposis J Clin Pathol: First Published As 10.1136/Jcp.47.9.860 on 1 September 1994

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86080 Clin Pathol 1994;47:860-861 Pancreatic islet cell tumour in a patient with familial adenomatous polyposis J Clin Pathol: first published as 10.1136/jcp.47.9.860 on 1 September 1994. Downloaded from

C J R Stewart, C W Imrie, A K Foulis

Abstract Schneider et al suggested that the numbers of A case of familial adenomatous polyposis endocrine tumours are significantly increased is reported in a 39 year old patient who in these patients.' We report a case of a man underwent a Whipple's resection for ade- with FAP who developed multiple adenomas noma of the duodenum. He subsequently of the duodenum, and was subsequently developed a pancreatic glucagonoma. found to have a pancreatic glucagonoma. Endocrine neoplasia other than papillary carcinoma of thyroid has seldom been reported in association with FAP. The Case report true incidence of islet celi tumours in A 39 year old man who had undergone total FAP may be underestimated. colectomy 20 years earlier for familial adenomatous polyposis presented with obstructive (3 Clin Pathol 1994;47:860-861) jaundice secondary to a large adenoma at the ampulla of Vater. Endoscopic sphincterec- tomy provided only temporary relief and, in It has long been recognised that familial view of the risk of carcinoma, the patient adenomatous polyposis (FAP) confers an underwent a Whipple's resection. The autosomal dominant susceptibility to the excised duodenal specimen included multiple development of multiple colonic neoplastic adenomas but none showed evidence of inva- polyps and colonic carcinoma. More recently, sive malignancy. A few months later the and with appropriate management of large patient presented with severe acute pancreati- bowel disease, the extracolonic manifestations tis which was complicated by the develop- of FAP have assumed greater importance. 1 2 ment of a pancreatic pseudocyst and a splenic Endocrine neoplasia other than papillary artery aneurysm. The aneurysm was resected carcinoma of thyroid has seldom been together with the spleen and the tail of pan- reported in association with FAP. However, creas leaving a segment of body of pancreas. tumours of adrenal cortex and pituitary have The patient remained well and non-diabetic been described, and a necropsy study by three years after the last operation. http://jcp.bmj.com/

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Department of Pathology, Glasgow 'a !-V' Royal Infirmary C J R Stewart A K Foulis Department of 'r r ILI Surgery

C W Imrie r *Aj,%V t. r Correspondence to: Dr C J R Stewart, Department of Pathology, Glasgow Royal Infirmary, Glasgow G4 OSF Accepted for publication Islet cell tumour is strongly reactive with glucagon antiserum. Immunoreactive cells are also evident in adjacent islets 24 March 1994 (immunoperoxidase). Pancreatic islet cell tumour in a patient withfamilial adenomatous polyposis 861

Pathological findings To our knowledge, only one previous case The specimen from the last procedure com- of islet cell tumour in a polyposis syndrome prised a 200 g spleen with a 12 x 8 x 7.5 has been reported (case 1 of the study by

cm encapsulated cystic lesion containing lam- Schneider et al).3 This patient, a man of 58, J Clin Pathol: first published as 10.1136/jcp.47.9.860 on 1 September 1994. Downloaded from inated thrombus at the hilum, and a 2 5 x was found to have a "benign non-function- 1-5 cm segment of pancreatic tail. The latter ing" islet cell tumour at necropsy after devel- was fibrotic and included on section a 1 1 x oping metastatic sigmoid carcinoma. The 0-8 cm circumscribed, focally haemorrhagic apparent rarity of islet cell tumours in FAP nodule close to one margin. may indicate that the association is coinciden- Histological analysis of the pancreatic nod- tal. The history of pancreatitis in our patient ule showed features typical of an islet cell may be relevant as islet cell tumours have tumour consisting of endocrine cells arranged been described in two patients with chronic in a trabecular or less commonly acinar pancreatitis.9 10 However, these cases differed pattern. Mild nuclear pleomorphism and in that the pancreatitis was of probable alco- occasional mitoses were identified. Immuno- holic aetiology and longer clinical duration. cytochemistry demonstrated diffuse tumour The true incidence of islet cell tumour in cell reactivity for glucagon (figure) and focal FAP may be underestimated as these staining with antisera to somatostatin and tumours may be clinically asymptomatic, as pancreatic polypeptide; there was no insulin in this case, and that previously described by immunoreactivity. Islets in the pancreatic Schneider and colleagues.' Furthermore, the parenchyma adjacent to the tumour gave the surgical prophylaxis of colonic carcinoma in expected mixed pattern of hormone affected patients may permit the development immunostaining. The parenchyma also of further previously unrecognised conditions showed features of obstructive pancreatitis. associated with FAP. The cyst at the splenic hilum was a false aneurysm of the splenic artery which was considered secondary to pancreatitis associated arterial wall damage. The adjacent 1 Parks TG. Extracolonic manifestations associated with familial adenomatous polyposis. Ann R Coil Surg Engl lymph nodes showed reactive changes while 1990;72:181-4. the splenic parenchyma was normal. 2 Jagelman DG. Extra-colonic manifestations of familial adenomatous polyposis. Oncology 1991;5:23-7. 3 Schneider NR, Cubilla AL, Chaganti RSK. Association of Discussion endocrine neoplasia with multiple polyposis of the colon. Cancer 1983;51:1171-5. Prevention of large bowel carcinoma in 4 Jagelman DG, DeCosse JJ, Bussey HJR. Upper gastroin- patients with FAP has resulted in increased testinal cancer in familial adenomatous polyposis. Lancet 1988;i: 1149-51. awareness of the non-colonic manifestations 5 Laferla G, Kaye SB, Crean GP. Hepatocellular and gas- of this condition. The risk of neoplasia in tric carcinoma associated with familial polyposis coli. Jf Surg Oncol 1988;38:19-21. other gastrointestinal sites, particularly the 6 Bombi JA, Rives A, Astudillo E, Pera C, Cardesa A. peri-ampullary region, is well recognised4 and Polyposis coli associated with adenocarcinoma of the gallbladder. Cancer 1984;53:2561-3. illustrated by the case presented here. 7 Spigelman AD, Farmer KCR, James M, Richman PI,

Patients with FAP are also known to have an Phillips RKS. Tumours of the liver, bile ducts, pancreas http://jcp.bmj.com/ and duodenum in a single patient with familial adeno- increased incidence of brain, bone, thyroid matous polyposis. BrJ Surg 1991;78:979-80. and adrenal tumours as well as desmoid dis- 8 Sohrabi AK, Nowzari P. Spectrum of clinical manifestations of familial adenomatous polyposis. W V Med J ease, epidermal cysts, and abnormalities of 1992;88: 193-4. retinal pigment epithelium." The spectrum 9 Allison MC, Renfrew CC, Webb WJS, Chappell ME, Pounder RE. Neuroendocrine islet cell tumour produc- of neoplasia in FAP continues to increase and ing gastrin and ACTH in a patient with calcifying recent reports have also documented associa- chronic pancreatitis. Gut 1985;26:426-8. 10 Prescot RJ, Manson J, Haboubi NH. islet cell Malignant on October 2, 2021 by guest. Protected copyright. tions with tumours of liver,5 gall bladder,6 bile tumour arising in chronic pancreatitis. Histopathology duct,7 and uterus and ovary.8 1993;22:499-501.