Multiple functioning paraganglioma associated with polycythemia.

Multiple retroperitoneal functioning paraganglioma complicated by polycythemia is reported in the case of a girl who, at the age of 13 years complained of headache, palpitation, and faintness preoperatively. In the retroperitoneal cavity, there were 21 paragangliomas larger than 1 cm in diameter. All were removed surgically in two sections, the first in 1979 and the second in 1983. Bilateral adrenals were macroscopically normal, and her symptoms disappeared. Now the recurrence of retroperitoneal paraganglioma is likely, although there have been no symptoms for 8 years, since the first operation. It is difficult to conclude that the tumors were benign or malignant in this case. Though complication of polycythemia had been diagnosed before the operation, the symptom continued even with the serum erythropoietin level kept in the normal range. Association of polycythemia with paraganglioma is extremely rare, and it is also rare to find more than 10 paragangliomas. Only a limited number of reports have been made on each case in the literature.