Abstract
Background: Paraganglionic neoplasms that originate in the adrenal medullas are referred to as pheochromocytomas, but if they arise from other paraganglia scattered throughout the body, they are referred to as paragangliomas. Pancreatic paragangliomas are an extremely rare entity as only 20 cases have been reported in the literature. They tend to be nonfunctional and typically occur in the fourth to fifth decade of life without a gender predilection. We describe in this study a case of a pancreatic paraganglioma and its CT appearance.Case Presentation: A 62-year-old woman undergoing presurgical evaluation for an olfactory groove meningioma resection was incidentally found to have a pancreatic mass. Multiple fine needle aspirations of the mass through endoscopic ultrasound yielded only atypical epithelial cells. The mass demonstrated avid enhancement on serial CTs with mild interval growth over a period of 5 years. No lymphadenopathy was ever found. The patient's complete blood count, complete metabolic panel, and plasma carcinoembryonic antigen levels were all within normal limits. Urine catecholamine metabolite levels were never checked as the patient demonstrated no symptoms of catecholamine excess. The patient underwent a laparoscopic distal pancreatectomy and splenectomy, and the mass was eventually diagnosed as a pancreatic paraganglioma through pathology. While the patient tolerated the surgery well, she did require a biliary sphincterotomy and placement of a pancreatic duct stent postoperatively for treatment of a pancreatic duct leak, which completely resolved. She showed no evidence of disease recurrence on multiple subsequent CTs and continues to do well.Conclusion: Pancreatic paragangliomas are usually incidentally discovered and typically demonstrate avid homogenous enhancement on contrast-enhanced CT or MR. Aggressive surgical resection is necessary to maximize the chances of disease-free survival. Pancreatic paragangliomas are similar histologically, whether benign or malignant, to paragangliomas that occur anywhere else in the body, with ∼70% in the abdomen and 30% in the chest.
Highlights
Introduction and BackgroundParaganglia are specialized cells derived from the neural crest that lie in close proximity to sympathetic ganglia throughout the body
Tumors originating in the adrenal medulla are referred to as pheochromocytomas, whereas if they arise from other paraganglia, they are referred to as paragangliomas
We describe in this study a case of a pancreatic paraganglioma
Summary
Paraganglia are specialized cells derived from the neural crest that lie in close proximity to sympathetic ganglia throughout the body. Components of the paraganglionic system include the adrenal medulla, the carotid, aortic and vagal bodies, and scattered ganglia throughout the chest and abdomen.[1] They are involved with producing rapid physiologic changes in the body through the production of catecholamines.[2] Tumors originating in the adrenal medulla are referred to as pheochromocytomas, whereas if they arise from other paraganglia, they are referred to as paragangliomas Their incidence is equal in both men and women, and they typically occur during the fourth to fifth decade of life.[1] These tumors can be functional and produce excess catecholamines in more than half the cases. Multiple repeat CT scans since the surgery, with the most recent one in September 2015, showed no evidence of recurrent disease or lymphadenopathy, and the patient continues to do well
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