Dr. J. Ravindra*, Deepthi Somarouthu


4D Syndrome is a term used to describe a manifestation of syndromic Glucagonomas, a type of Pancreatic endocrine tumor. According to WHO classification of tumors of the digestive system, glucagonoma is a type of functional pancreatic neuroendocrine neoplasm(pNEN) This pancreatic neuroendocrine tumor(pNET) secretes glucagon and causes a combination of symptoms known as glucagonoma syndrome. Glucagonoma is an extremely rare pancreatic alpha-islet cell tumor and is often accompanied by certain clinical symptoms including necrotizing migratory erythema (NME), diabetes, weight loss and anemia.[3] In 75-80% of cases, the glucagonoma starts in malignant form, and in 50% of these cases, metastasis exsists at diagnosis. Diagnosis is elevated glucagon levels. Tumor is localized with CT and endoscopic ultrasonography.[5] The treatment include where the tumor is, its size and whether it has spread(stage). Surgery includes thev removal of –just the tumor(enucleation), the narrowest part of the pancreas and the body of the pancreas(distal pancreatectomy), the whole of the pancreas(total pancreatectomy), the widest part of the pancreas, duodenum, gallbladder and part of the bile duct(pylorus preserving pancreaticoduodenectomy or PPPD for short), the widest part of the pancreas, duodenum, gallbladder, part of the bile duct and part of the stomach(whipple s operation). Some drugs cause partial regression of NME, In the literature good results have been obtained with streptozotocin+/doxorubicin or 5-fluorouracil, Sunitinib was approved by FDA for PNET, Lanreotide(somatuline depot) has been given for metastatic gastroenteropancreatic neuroendocrine tumors(GEP-NETs), to improve progression – free survival.[19]

Keywords: Glucagonoma, pNEN, pNET, GEP-NET, Enucleation.

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