Contributed by Yan Peng MD, PhD and Sydney Finkelstein, MD, PhD
Published on line in August 2000
A 71-year old white woman became aware of "fainting spells" which included dizziness, feeling of warmth and headaches. These spells occurred suddenly without warning several times each day. She had no family history of endocrine problems. Physical examination revealed moderate hypertension. A 24 hour urine collection showed marked accumulation of normetanephrine with a level of 12,751units (normal less than 350 units), and a moderate elevation in metanephrine with a level of 578 unites (normal less than 350 units). Her epinephrine, norepinephrine and dopamine levels, and stimulated cortisol and aldosterone levels were within normal limits. A 24 hour free cortisol was not performed. A CT scan and a MRI of the abdomen revealed a 4 cm mass in the region of the left adrenal gland. A fine needle biopsy of the mass showed adrenal medullary tissue staining focally positive for chromogranin. The patient underwent a left adrenalectomy with complete removal of the mass. Her blood pressure returned to normal after operation and her symptoms disappeared.
Microscopic examination of material subjected to formalin fixation, paraffin embedding and hematoxylin and eosin staining revealed two types of the cells in the tumor ( IMAGE 01). One component had round-oval nuclei with coarsely clumped chromatin and eosinophilic granular cytoplasm (IMAGE 02). Another component had clear cytoplasm (IMAGE 03). The tumor also showed areas with thrombosis and recanalization of vascular channels (IMAGES 04 and 05).
Immunostaining for chromogranin (IMAGE 06), synaptophysin (IMAGE 07) and neuron specific enolase (IMAGE 08) was positive for the pheochromocytoma cells. Immunostaining for S-100 protein showed the sustentacular cells at the peripheries of the tumor cell clusters (IMAGE 09).
Electron microscopic examination showed that the tumor cells contained neuroendocrine granules and smooth endoplasmic reticulin (IMAGES 10 and 11).