MALIGNANT COMPOSITE PHEOCHROMOCYTOMA OF THE ADRENAL GLAND IN A PATIENT WITH VON RECKLINGHAUSEN’S DISEASE

Abstract

A 59-year-old woman with von Recklinghausen’s disease was referred to us in February 2000 with a palpable abdominal mass in the right upper quadrant portion (fig. 1). On hospital admission the patient was poorly nourished (weight 36 kg., height 144 cm.). Pulse rate was 98 beats per minute and blood pressure was 198/118 mm Hg. Electrolytes, and renal and liver function were normal. Urinalysis demonstrated proteinuria and glycosuria. Endocrine examination revealed that plasma norepinephrine was 12.63 ng./ml. (normal 0.07 to 0.32), urinary norepinephrine 4,470 mg. per day (normal 10 to 41) and urinary vanilly mandelic acid 98.2 mg. per day. Serum neuron-specific enolase was greater than 200 ng./ml. (normal less than 10). The patient had no symptoms of diarrhea and hypocalcemia due to vasoactive intestinal polypeptide, which was not measured. Computerized tomography and magnetic resonance imaging showed a 10 3 6 cm. mass in the right adrenal gland, which was compressing the liver, and consisted of cystic components (fig. 2). Multiple lung and bone metastases were also detected. The abnormal accumulations in these metastatic lesions could not be confirmed on 131 iodine metaiodobenzylguanidine scintigraphy. The patient rapidly became emaciated and died 3 months after hospital admission. An autopsy of only the tumors in the right adrenal gland and bone with metastasis was permitted. Gross examination revealed that the tumor was of cystic degeneration with necrotic or hemorrhagic portions. Histologically, the primary tumor was predominantly composed of solid nesting or Zellballen proliferation of polygonal tumors cells with round or oval nuclei and prominent nucleoli, which had an eosiophilic finely granular cytoplasm. Scattered large, bizarre nuclei and a few multinucleated tumor cells were also present (fig. 3, A). Among these typical patterns of pheochromocytoma, the small foci of neuroblastoma characterized by mildly elonAccepted for publication November 22, 2000. * Requests for reprints: Department of Urology, Kochi Medical School, Nankoku, Kochi, Japan 783-8505.