[An autopsy case of malignant retroperitoneal paraganglioma]

A case of an extra-adrenal malignant paraganglioma of the retroperitoneum is reported. A 33-year-old woman with a complaint of a dull pain in the right thigh was admitted. A CT scan revealed a large retroperitoneal mass situated between the right kidney and the aorta. A biopsied specimen of the right femur uncovered a metastatic focus of a paraganglioma. Subsequent chemotherapy and irradiation ultimately proved ineffective, and the patient died at the age of 37. Autopsy revealed a retroperitoneal mass 11 X 9 X 4 cm, and its metastatic foci in the vertebrae, ribs, sternum, and liver. Six cases of extra-adrenal malignant paragangliomas of the retroperitoneum that have been reported in Japan also are briefly reviewed.