PHEOCHROMOCYTOMA OF THE ADRENAL GLAND WITH GRANULOSA CELL TUMOR AND NEUROFIBROMATOSIS: REPORT OF A CASE WITH FATAL OUTCOME FOLLOWING ABDOMINAL AORTOGRAPHY

Abstract

A case of pheochromocytoma of the left adrenal gland associated with malignant granulosa cell tumor and neurofibromatosis of von Reckling-hausen is reported. Pheochromocytoma has not previously been reported associated with malignant granulosa cell tumor but has been reported in cases of neurofibromatosis in 18 previous occasions. A search of the literature discloses two other cases of death following abdominal aortography in patients with pehochromocytoma. Extensive periadrenal bleeding was found in both cases as in the one herein reported, emphasizing the danger of this procedure in the presence of such adrenal tumors. The mechanism of death appears to be adrenalinic crisis precipitated by intraadrenal and/or periadrenal hemorrhage, with liberation of large amounts of pressor substances (epinephrine and norepinephrine). In the case herein reported, the vasopressor substances were determined by Von Euler''s colorimetric method. In the adrenal tumor, the total vasopressor substances were 22.2 mg/g of tumor tissue or a total of approximately 1650 mg. In the heart, the total catechol was 1.46 [mu]g/g of heart tissue (the arbitrary upper limit being 0.6 [mu]g/g of heart tissue).