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Recurrent Insulinoma Syndrome with Metastatic Glucagonoma

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Recurrent Insulinoma Syndrome with Metastatic Glucagonoma J Clin Pathol: first published as 10.1136/jcp.36.9.1076 on 1 September 1983. Downloaded from J Clin Pathol 1983;36:1076-1080 Recurrent insulinoma syndrome with metastatic glucagonoma PJS DUNN*, MC SHEPPARDt, DA HEATHt, G SLANEYt From the Departments of *Pathology, tMedicine and tSurgery, University of Birmingham, Queen Elizabeth Hospital, Birmingham B15 2 TH SUMMARY A case is reported of a patient who presented with symptomatic hypoglycaemia and who had three pancreatic tumours resected over the ensuing eight years. Immunocytochemistry demonstrated two of these to be insulinomas and the third to be a glucagonoma. In addition metastatic spread of cells positive for glucagon had occurred to a lymph node and multiple nodules staining positively for glucagon were present in the remainder of the pancreas. Pancreatic endocrine tumours are uncommon but of by a pancreatic pseudocyst but he remained normog- great interest because of the variety of clinical syn- lycaemic, even after a 24-hour fast. dromes which they produce. Sensitive After six asymptomatic years without treatment immunocytochemical studies have shown that many he again presented with hypoglycaemia and inap- of these tumours are composed of several cell types propriately raised serum insulin concentrations (glu-copyright. and are multihormonal.4 5 0 cose 2-9, 1-5 mmoL/l, insulin 35, 38 mU/l). He was Transformation from one syndrome to another treated with diazoxide 100 mg tds. Transhepatic has been reported9 and presumed to be due to portal vein sampling revealed a large "step-up" in change in type of the dominant cell in a mixed serum insulin at the level of the head of pancreas. At tumour. We report a case of recurrent insulinoma surgery (1981) a 2*5 cm tumour was removed from syndrome with metastatic glucagonoma, shown by the head of the pancreas. Immediately postopera- immunocytochemistry to be due to the coexistence tively his blood glucose rose to within the normal of insulinoma and glucagonoma. The importance of range but on the second postoperative day it in immunocytochemistry accurately defining the returned to the preoperative concentration and he http://jcp.bmj.com/ nature of these tumours is emphasised. subsequently required continued diazoxide treatment. Repeat transhepatic portal vein sampling Case report confirmed persistently high insulin concentrations in veins draining the head of pancreas and selective A 24-year-old caucasian man (date of birth 7.8.58) arteriography demonstrated a tumour in the head. presented in June 1974 with a grand mal convulsion, Further surgery (1982) was undertaken and hypoglycaemia and inappropriately raised serum another tumour was identified in the head of pan- on October 3, 2021 by guest. Protected insulin concentrations (corresponding glucose and creas adjacent to the superior mesenteric vein. A insulin values were 1*7, 2*3, 1-7, 1*7 mmolI and 39, pancreatico-duodenectomy was performed. Post- 38, 32, 20 mU/l respectively). In addition mild operatively the patient has remained asymptomatic hypercalcaemia was noted (2-88 mmol/1) which was and normoglycaemic with no treatment. unresponsive to hydrocortisone administration. Raised parathyroid hormone (PTH) concentrations Material and methods were subsequently documented and his father was noted to have mild hypercalcaemia with high PTH Multiple sections of formalin-fixed paraffin- concentrations, suggesting a multiple endocrine embedded tissue from all three surgical specimens adenopathy syndrome, type I. At laparotomy in were examined using haematoxylin and eosin, August 1974 a tumour in the tail of the pancreas was aldehyde fuchsin, congo red and lead haematoxylin removed. His postoperative course was complicated stains and by the Grimelius silver impregnation technique.' In addition serial sections from the same Accepted for publication 27 April 1983 blocks were examined by means of the PAP method 1076 J Clin Pathol: first published as 10.1136/jcp.36.9.1076 on 1 September 1983. Downloaded from Recurrent insulinoma syndrome with metastatic glucagonoma 1077 'A) ' W\,v b nsa Fig. 1 Tumour removed from tail ofpancreas in 1974. Fig. 2 Tumour removed from head ofpancreas in 1981. Immunocytochemical demonstration ofinsulin in the Immunocytochemical demonstration ofglucagon in the majority of tumour cells. Unlabelled antibody-enzyme majority oftumour cells. Unlabelled antibody-enzyme method using guinea-pig anti-insulin antiserum as first method using rabbit antiglucagon antiserum as first layer. layer. x 575. x 575. of Stemberger et al,2 after treatment with trypsin,3 were unstained by aldehyde fuchsin or lead using antisera to insulin, glucagon, and somatosta- haematoxylin. No amyloid was demonstrable. tin. The antisera were used at the following dilu- Immunocytochemistry showed the majority of cells tions: guinea pig anti-insulin (Immuno-nuclear to contain insulin-positive granules (Fig. 1) with copyright. Corp., PO Box 285, Stillwater, Minnesota, USA) occasional cells staining for somatostatin. The two 1/500; rabbit antiglucagon (Mercia Brocades, cell types appeared distinct in serial sections Brocades House, Pyrford Road, West Byfleet, Sur- examined. No positive staining for glucagon was rey) 1/500; rabbit antisomatostatin (Immuno- observed in any of the sections examined. A few nuclear Corp., PO Box 285, Stillwater, Minnesota, cells failed to react with any of the antisera used. USA) 1/800. The second and third stages were per- formed using sheep antirabbit (RIA UK, 3 Manor 1981 Place, Athenaeum Street, Sunderland) and rabbit The second tumour removed was a cystic mass 2 cm PAP complex (Mercia Brocades). Appropriate con- x 2 cm x 1*5 cm containing straw-coloured fluid. A http://jcp.bmj.com/ trols were performed, using the specific antibodies small amount of exocrine pancreatic tissue was after prior absorption with an excess of the corres- attached at one pole. Microscopy showed similar ponding antigen as a first layer, non-immune serum cells to the first tumour arranged in a gyriform pat- as first layer, and by omission of 3,3'diaminoben- tern around numerous blood vessels. A thin capsule zidine tetrahydrochloride from the incubation of hyaline collagen was present, with areas of medium for the peroxidase reaction. infiltration by tumour. Mitoses were not seen. Routine histological staining reactions were identi- on October 3, 2021 by guest. Protected PATHOLOGICAL FINDINGS cal to the 1974 tumour but the immunohistochemi- The findings are described separately for the three cal reactions were quite distinct. No positive staining tumours, identified by the year of removal. was obtained with anti-insulin or antisomatostatin, but almost all the tumour cells showed moderate to 1974 strong staining with antiglucagon (Fig. 2). This was a 2 cm diameter ovoid mass with no attached pancreatic tissue. Microscopy showed a cel- 1982 lular tumour composed of irregular sheets and broad This specimen consisted of pylorus, duodenum, and trabeculae of cuboidal basophiic cells with large the head of the pancreas. Within the head of the uniform open nuclei. Occasional mitotic figures pancreas and extending to the anterior border there were observed. The cells were arranged around a was a firm congested clearly defined nodule 2 cm rich sinusoidal vascular network and a thin fibrous diameter. The pancreatic tissue was otherwise capsule was present. Many cells showed silver- unremarkable macroscopically. Several small positive granules by the Grimelius technique but peripancreatic lymph nodes were identified. Micros- J Clin Pathol: first published as 10.1136/jcp.36.9.1076 on 1 September 1983. Downloaded from 1078 Dunn, Sheppard, Heath, Slaney Fig. 3 Main tumour removed with head ofpancreas in 1982 showing gyriform pattern. Haematoxylin and eosin. x 92. Fig. 5 Metastatc deposit in peripancreatic lymph node removed with head ofpancreas in 1982. Haematoxylin and eosin. x 92. .4 :I q:k ..I copyright. * C,- w ~~~~.. http://jcp.bmj.com/ Fig. 4 Main tumour removed with head ofpancreas in 1982. Immunocytochemical demonstration ofinsulin in the majority of the tumour cells. Unlabelled antibody-enzyme method using guinea-pig anti-insulin antiserum as first layer. x 575. Fig. 6 Metastatic deposit in peripancreatic lymph node on October 3, 2021 by guest. Protected copy of the tumour showed identical appearances to removed with head ofpancreas in 1982. Immunocytochemical demonstration ofglucagon in all of the previous specimen apart from the absence of a the tumour cells. Unlabelled antibody-enzyme method using clearly defined capsule (Fig. 3). Silver-positive rabbit antiglucagon as first layer. x 230. granules were again demonstrable and aldehyde fuchsin and lead haematoxylin were negative. tical to the main tumour. Three of the six were Immunocytochemistry showed the majority of cells encapsulated. A further area of tumour was to contain insulin (Fig. 4) with a few staining posi- identified within the subcapsular sinus of a peripan- tively for somatostatin. No glucagon-containing cells creatic lymph node (Fig. 5). All six nodules and the could be identified in multiple sections examined. lymph node metastasis were strongly positive for Examination of tissue from elsewhere in the glucagon (Fig. 6) and negative for both insulin and excised pancreas revealed six discrete irregular somatostatin. endocrine cell nodules varying in size from 0 1 cm to The pancreatic islets in the intervening pancreas 0-5 cm. The cellular morphology of all six was iden- were irregular in outline and many were hyperplas- J
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