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home - Pancreas - Pancreatic Cancers - Gastrinoma and Glucagonoma Written by Dr Sebastian Zeki

Knows the presentation investigation and staging of pancreatic

Recognises the importance of considering and being able to identify
uncommon pancreatic tumours (such as neuroendocrine or
intrapapillary mucinous tuours)

Knows the range of potential therapies and recognises the factors
that make such tumours potentially operable or inoperable

Knows the prevalence and natural history of benign cysts/serous
cystadenoma and potentially malignant cystic lesions

Knows the options for palliative treatment
Shows ability to sequence investigations appropriately
Understands value of multi-disciplinary team
Recognises the importance of considering possibility that the tumour
is unusual

Communicates effectively within the multi-disciplinary team and with
the patient and their family

Gastrinoma and Glucagonoma

EpidemiologyThe average age of onset is 40-50 years old.It has an equal sex incidence.It is rarely associated with (MEN 1). Glucagonoma and the glucagonoma syndrome Gastrinoma DiagnosisIn patients with a suspected gastrinoma, SPECT imaging with pentetreotide be the first test .Octreotide scan sensitivity for gastrinomas= 58 %, (=other imaging studies (ultrasonography, CT (sensitivity of 40%), MRI, and angiography) combined).If no tumour is found, but clinical suspicion remains high, we suggest EUS be performed to locate the primary tumour.If a primary tumour is found, we suggest a MRI or helical CT to exclude hepatic metastases.Can also give secretagogues into arteries supplying pancreas (secretin for gastrinomas) with subsequent sampling of the hepatic venous effluent.This is more accurate than portal vein sampling although the role is not yet defined.If EUS does not identify a primary tumour but suspicion remains strong, we suggest angiography or surgical exploration intraoperative palpation, duodenal transillumination and ultrasonography. Gastrinomas Prognosis- There is a 50% 5 year survival. PathologyThe disease is of the alpha cells of the pancreas.They are usually encapsulated, firm nodules, varying in size from 2 cm up to 25 cm.Lesions are most often in the tail of the pancreas.Lesions are usually slow growing.Lesions have usually metastasised by diagnosis.Histologically cells look like cords/ nests of alpha cells 2-25cm alpha Clinical ManifestationsWeight loss and NME are the most prevalent symptoms, occurring in 70 % by diagnosis .Necrolytic migratory erythema — Diabetes mellitus occurs in 75%.Abdominal pain, anorexia, diarrhoea, and constipation can occur.Venous thrombosis occurs in 30%.Neurologic symptoms can occur - eg. ataxia, dementia, optic atrophy, and proximal muscle weakness. Lab Results:A raised glucose and glycogen.A normochromic, normocytic anaemia.Decreased aminao acids (?causes NME).A raised serum glucagon —Usually >500pg/mL. Imaging techniques:CT- sensitivity approaches 100% as usually lesions >3cm.EUS with biopsy and Immunoperoxidase staining/ glucagon mRNA.Angiography - usually not needed.Somatostatin receptor scintigraphy using radiolabeled octreotide-usually unnecessary. Resection — Cure rate of 30% Begins as erythematous papules or plaques involving the face, perineum, and extremities . Over the next 7-14 days, the lesions enlarge and coalesce. Central clearing then occurs, leaving bronze-colored, indurated areas centrally, with blistering, crusting, and scaling at the borders.The affected areas are often pruritic and painful.The same process often affects the mucous membranes, resulting in glossitis, angular cheilitis, stomatitis, and blepharitis. Hair thinning and dystrophic nails are also commonly observed. Written by Dr Sebastian Zeki

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