A Case of Primary Paraganglioma that Arose in the Pancreas: the Color Doppler Ultrasonography and Dynamic CT Features

Abstract

Paragangliomas rarely originate from the pancreas and they are characterized on imaging studies as well-marginated, hypervascular masses with cystic areas. We herein report on a case report of pancreatic paraganglioma in a 57-year-old woman, which was confirmed on pathology. Color Doppler ultrasonography and dynamic CT demonstrated a well-demarcated, extremely hypervascular mass with prominent intratumoral vessels and early contrast filling of the draining veins from the mass. Endoscopic retrograde cholangiopancreatography showed that the main pancreatic duct was displaced and mildly dilated.

Paraganglioma de pâncreas: relato de caso e revisão da literatura

“…Other less common locations for abdominal paragangliomas include gallblader, urinary bladder, prostate, spermatic cord, uterus, and duodenum. Paraganglioma of the pancreas is extremely rare, and only 14 cases, including the present one, have been reported worldwide 2,3,4,56,7,8 . When the diagnosis of paraganglioma is made, it's necessary screening other tumours, because until now this type of neoplasm has been considered part of multiple endocrine neoplasia syndrome, secundary of genetic alteration predisposing presence of multiple tumors 1 .…”

“…The diagnosis was established by imaging exams revealing a well-defined mass with frequent areas of hypoechogenicity in ultrasonography, a well-marginated, hypervascular tumor with cystic areas of low-attenuation on contrast-enhanced CT 3,8 . Smaller tumors are more likely to show homogeneous attenuation and they can be sharply marginated, as compared to larger ones, demonstrating a rupture of vascular trauma due to the neoplasia and tumoral necrosis 4,6,8 .…”

Retroperitoneal Paragangliomas in Obese Patients

“…They can affect the adrenal medulla, carotid body, jugular foramen, middle ear, aorticopulmonary region, posterior mediastinum, organs of Zuckerkandl, abdominal paraaortic region, cauda equina, and other unnamed paraganglia occurring in the distribution of the sympathetic and parasympathetic nerves [4,5]. Other less common locations of abdominal paragangliomas include the gallbladder, duodenum (gangliocytic paraganglioma), pancreas, urinary bladder, prostate, spermatic cord, and uterus [5,7].…”

“…Paragangliomas are rare endocrine tumors that arise from paraganglionic tissue, affecting about one in 2,000,000 people [7]. The mean age at diagnosis is 47 years old (adults in the fourth or fifth decade of life), and there is no sex predilection [4,5].…”

A Rare Case of Pancreatic Paraganglioma: Histological and Immunohistochemical Aspects

“…Clinical incidences have been estimated 1.5 per million per year in the case of paragangliomas. [4] Pancreatic paragangliomas are generally non-functional, [5] in contrast to tumors arising from other sites, where functional activity is more common. In tumors associated with symptomatic hypertension, headache, palpitations, and sweating, catecholamine secretion is reported in 30-60% of cases.…”