Paragangliomas are rare neuroendocrine tumors, which arise from the extra-adrenal chromaffin cells. They are found in the tissues such as the adrenal medulla, carotid and aortic body, organs of Zuckerkandl, and paraganglia of the sympathetic and parasympathetic neurons. They represent 10-18% of all chromaffin tissue related tumors. The head, neck, and retroperitoneum are the most common sites for paragangliomas. Other less common locations for abdominal paragangliomas include the gallbladder, urinary bladder, prostate, spermatic cord, uterus and duedonum
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Histologically, all paragangliomas present a similar apperance regardless of their site of origin. They consist of clusters of cells separated by a highly vascular, reticular network forming the characteristic zellballen pattern. The malignant potential of these tumors cannot be determined from their histological appearance 3, 4.
Paraganglioma of the pancreas is also rare and only 14 patients including our case have been reported up to now 1, 3, 5-12 (Table 1). Eight cases were considered to be benign from pathological and clinical findings, while the frequency of malignant paragangliomas of the retroperitoneum ranged between 20% and 42% 3, 10, 13. The mean age of these 14 cases was 63 years. the male to female ratio was 0,75/1. In the nine patients, the tumor was located in the head of the pancreas. In the three cases, tumors were located in the corpus of pancreas and in the one, it was located in the tail of pancreas. In our case, tumor was located in the corpus of pancreas.
Paraganglioma, like other benign tumors, usually does not present any symptoms (espacially nonfunctional tumors) and is often found incidentally 11, In ous case, presented epigastric pain. In functional tumors, urinary catecholamines are elevated, usually with predominance of norepinephrine 1, 6. Abdominal ultrasonography and CT scan generally demonstrate a well defined mass. Paragangliomas are characterized by highly vascular and well-enhanced tumors with a cystic area in CT scan 3.
The main therapy is surgical resection. In general, the resection of paraganglioma of the pancreas is technically difficult due to the anatomical complexity around the pancreas and the possibility of sudden catecholamine release during the operation 10. In our case, tumor was resected. The local resection was performed in 9 of the 14 patients in the literature. Pancreatectomy was the another resection procedure used in 4 of the 14 patients. All of these patients showed equally good outcome after surgery. Our case is still in complete remission also.
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