Malignant abdominal paraganglioma with lymph node metastasis: report of a case

BACKGROUND: Paragangliomas are rare tumors that generally occur in the abdominal paraaortic area down to the aortic bifurcation. Diagnosis is often made incidentally during radiologic work-up for abdominal pain. As benign and malignant lesions have identical clinical, radiologic and histologic features, the malignant potential is determined by local invasion and distant metastases. METHODS: We report a 49-year-old male who underwent lymphadenectomy for a retroperitoneal tumor with a largest dimension of 6.5 cm above the abdominal aorta, which was diagnosed by CT. RESULTS: Histopathologic resection of the specimen showed malignant paraganglioma with lymph node metastasis. The operative and postoperative course was uneventful. CONCLUSIONS: Malignant abdominal paragangliomas are rare tumors whose clinical, radiologic and histologic presentations are identical to the benign form; only the presence of local invasion or distant metastases confirms malignancy. The treatment of choice is complete surgical resection. Only a multidisciplinary approach will allow optimal treatment for this selected group of patients.