Abstract
BackgroundPancreatic paragangliomas are rare tumors, with only 16 reported cases to date. One of these cases demonstrates metastasis to lymph node, while another case was functional, however, none of these cases showed malignant and large, pancreatic paraganglioma with marked invasion. Also another unique feature was the age of our patient compared to the average reported ages in published literature (42–85 years).Case presentationA 19-year-old woman presented with a one-year history of intermittent abdominal pain. Physical examination showed a palpable mass in the right upper abdomen, but initial laboratory results were within normal ranges; tumor markers (CEA, AFP, and CA19-9) were negative. An abdominal and pelvic computed tomography (CT) scan showed a well-defined retroperitoneal para-aortic mass. The CT scan revealed that the surrounding lymph nodes were not enlarged, but the liver showed evidence of parenchymal infiltration. Intraoperatively, a large, firm tumor originating from the head of pancreas was found pushing on the caudate hepatic lobe and the inferior vena cava (IVC). The tumor was resected through a pancreaticoduodenectomy, involving segment VI of the liver and a small segment of the IVC. The blood pressure spiked (>220 mm Hg) when the tumor was manipulated during the operation. The final pathology report showed a 9-cm tumor with lymphovascular invasions; immunohistochemistry was positive for synaptophysin and chromogranin. All resection margins were negative and 1/15 lymph nodes was positive for metastasis. Post-operative recovery was unremarkable. One month after discharge, the patient was re-admitted with abdominal pain and found to have an abdominal collection at the resection site, which was drained under CT guidance. She received a therapeutic dose of I131-metaiodobenzylguanidine (MIBG). Follow-ups showed the absence of recurrence, and she has remained disease free.ConclusionThis patient was an extraordinary example of a rare tumor. Even more remarkable was that the tumor was malignant with lymph node invasion. To our knowledge, a case similar to that presented here has not been previously reported in the literature.
Highlights
Pancreatic paragangliomas are rare tumors, with only 16 reported cases to date
We report here a case of pancreatic paraganglioma, was identified through computed tomography (CT) scan and histological evidences
We ruled out that the tumor was retroperitoneal because it was not attached to any structure and it fully mobilized with the head of the pancreas on kocherization, as a single mass
Summary
We report here a case of pancreatic paraganglioma, was identified through CT scan and histological evidences. The patient was not jaundiced and her abdomino-pelvic CT scan showed a well-defined, hypervascular pancreatic mass with evidence of hepatic parenchymal infiltration, but a normal biliary tree. Hypervascularity is another common feature of paragangliomas of the pancreas and other sites [6]. Pheochromocytomas and extraadrenal paragangliomas have unpredictable behavior and often metastasize late These tumors should never be considered malignant unless there is documented metastasis to the lymph nodes or other distant sites. Author details 1Department of Surgery, College of Medicine and Medical Sciences, Taif University, PO Box 888, Taif 21947, Kingdom of Saudi Arabia. 2Department of Surgery, Al-Hada Military Hospital, PO Box 1347, Taif, Kingdom of Saudi Arabia. 3Department of Pathology, Al-Hada Military Hospital, PO Box 1347, Taif, Kingdom of Saudi Arabia
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