Abstract
IntroductionComposite paragangliomas consist of two components, paraganglioma and ganglioneuroma, representing a rare subgroup of paragangliomas. The purpose of the study is to describe a case of composite paraganglioma of the celiac trunk and a brief review of the existing literature.Case PresentationA 64-year-old female patient with a history of epigastric abdominal pain and a 51 mm-diameter tumor found in a Computerized Tomography of the abdomen was admitted to our surgical department for further evaluation and treatment. After a brief preoperative surgical assessment, the patient underwent a mini-laparotomy for the excision of this tumor. After having the results of the pathology report, a comprehensive review of the international literature was carried out by applying the appropriate search terms.ResultsAs it was found intraoperatively, the tumor was located at the cephalad aspect of the common hepatic artery, over the portal vein and the inferior vena cava. A negative-margin resection was achieved and the tumor was sent for pathology analysis. The final pathology report revealed a composite paraganglioma, with α paraganglioma and a ganglioneuroma component. Seventeen cases of extra-adrenal composite paraganglioma have been reported in the international literature so far. This case was the first one found in the area of the celiac trunk.ConclusionsComposite paragangliomas comprise rare and potentially malignant tumors with variable prognosis. Establishing their diagnosis promptly is of vital significance. Due to the first-described location of the composite paraganglioma in our case, the differential diagnosis of tumors in this area should also include composite paragangliomas.
Highlights
Composite paragangliomas consist of two components, paraganglioma and ganglioneuroma, representing a rare subgroup of paragangliomas
We present a case of composite paraganglioma located on the right of the celiac trunk, over the common hepatic artery
In our case, since the composite paraganglioma was not an active one, the patient did not experience any of the relating with catecholamines-secretion symptoms, but only a feeling of abdominal tenderness and pain especially right after exercise
Summary
Composite paragangliomas consist of two components, paraganglioma and ganglioneuroma, representing a rare subgroup of paragangliomas. The purpose of the study is to describe a case of composite paraganglioma of the celiac trunk and a brief review of the existing literature. Pheochromocytomas are rare chromaffin catecholaminesecreting tumors, usually located within the adrenal glands. When these tumors arise outside of the adrenal glands, they are defined as paragangliomas. Composite paragangliomas are estimated to be around 3% of the adrenal paragangliomas, being very rare especially outside the adrenal glands [1] They are reported to be found usually in the adrenal glands and less frequently in the mediastinum, in the Zuckerkandl organ at the abdominal aortic bifurcation, in the retroperitoneum, in the urinary bladder, and the central nervous system [2, 3]. We present a case of composite paraganglioma located on the right of the celiac trunk, over the common hepatic artery
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