An extra-adrenal paraganglioma treated with radiotherapy

Abstract

Abdominal paragangliomas (PG) are usually misdiagnosed as retroperitoneal masses originated from pancreas, kidney or adrenal gland. Although the optimum treatment of the disease is surgical excision, resection may not be proper secondary to advanced disease or close relationship with the main vessels.Case:A 72 year old female patient was suffering from right lumbar pain and feeling of fullness for last 1 year. In another medical center a computed abdomen tomography (CT) had been carried out which was showing a pancreatic mass. For further evaluation she was referred to our clinic. Physical examination revealed bilateral grade 2 lower extremity edema and an appendectomy scar. We repeated CT and performed three-dimensional reconstruction revealing of a 35x52x42 mm sized hypervascular mass which was separate from the pancreas. This mass was partially surrounding inferior vena cava and entering the aorta-caval groove. CT-guided trucut biopsy was performed. Pathology resulted as benign, low mitotic activity PG. She was diagnosed as extra-adrenal paraganglioma (EAPG). Being close to the main vessels, instead of surgical excision radiotherapy (RT) was decided. After completion of the treatment, abdominal pain and lower extremity edema disappeared. 6 months after RT on control CT, there was no progression in the size of the mass.PG is a rare tumor. Because of the risk of malignant transformation, decided treatment should be surgery. After confirmation of the diagnosis, for paragangliomas which has close relationship to main vessels RT is an alternative treatment.