Pancreatic Gangliocytic Praganglioma: A Rare Case and Literature Review

Abstract

Abstract Introduction/Objective Gangliocytic paraganglioma, GP is a morphologically distinct tumor first reported by Dahl in 1957, most often in second portion of duodenum near ampulla of Vater. GP mostly present as single tumors, but occasionally may be multiple, when seen in association with Von Recklinghausen disease and carcinoid tumors. Rare cases have been reported at other sites in gastrointestinal tract including esophagus, pylorus, jejunum and extra- gastrointestinal tract in the nasopharynx, thymus, lung and ovary. Pancreatic GP is extremely rare with five cases reported till date. Methods/Case Report A 42- year- old male presented with abdominal pain. Physical exam showed no abnormalities. The significant findings on labs were raised amylase levels in the range of 1262 U and mild hyperglycemia with blood glucose of 178mg/dl. CA 19-9 was found to be 8.0 U/ml. Coronal CT Abdomen showed hypodense well circumscribed nodule 1.5 cm in body of pancreas. Gross specimen of pancreas showed a circumscribed 1.5 cm nodule in body of Pancreas.The microscopic sections of pancreatic mass show epithelioid appearing neuroendocrine cells in nests, with spindle shaped schwann cells or sustentacular cells in periphery, and scattered ganglion cells. The immunohistochemical staining positive for synaptophysin in epithelioid cells, S100 in spindled schwann cells and CK in epithelioid cells and Ganglion cells. Based on the microscopic findings, a diagnosis of Gangliocytic Paraganglioma, GP arising in Pancreas was made. Results (if a Case Study enter NA) NA Conclusion Pancreatic GP, are often misdiagnosed as Pancreatic Neuro endocrine tumors, Grade1. The accurate differentiation and precise diagnosis is crucial for appropriate clinical management. WHO classification of 2010, classifies gangliocytic paragangliomas as benign. Duodenal GP has a benign behavior with lymph node metastasis in only 5-7% cases, and no evidence of distant metastasis. In comparison, Pancreatic GP show an more aggressive behavior with lymph node metastasis observed in 2/6 cases and one case with sternal bone metastasis, emphasizing the importance of the primary location of origin of GP as prognostic factor. Our understanding is limited due to the scarcity of literature; more published data shall be instrumental in further explaining the clinical behavior of this rare entity.