Extrarenal renin-secreting tumor associated with hypertension

Described herein is an autopsy case of a 16-year-old female with severe hypertension, hyperreninemia and secondary aldosteronism. She had had a progressively growing tumor of her right orbita from the age of 4. The tumor was partially excised 13 months before death. A high content of a renin-like material was detected in the excised tumor, which was histologically a hemangiopericytoma. Bowie stain revealed some granules in small number of tumor cells and electron microscopic study showed some cytoplasmic granules. Following the operation, hypertension was somewhat improved, but the levels of plasma renin activity and plasma aldosterone concentration remained elevated, because the tumor was partially resected. At autopsy, the tumor invaded into the cranial base and right frontal lobe, and metastasized to the lungs. In the present case, renal renin-secreting tumor, malignant hypertension and renovascular hypertension were ruled out by the clinical and pathological studies.