The association between medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism in various combinations has been referred to as multiple endocrine neoplasia type II (MEN II). We report here an unusual patient with a malignant pheochromocytoma and medullary thyroid carcinoma.
Case ReportThe patient was a 36-year-old Saudi male living in the desert near Al-Wajhe in the Northern Province who presented to King Faisal Specialist Hospital in March 1984 with a 10-month history of tremor, headache, vomiting, dizziness, anorexia, postprandial epigastric pain, 20-kg weight loss, dysphagia, and hoarse voice. His five brothers and four sisters were reported to be in good health. Specifically, none of them had a history of hypertension or goiter. Numerous attempts to persuade the family to come to our hospital for a study were unsuccessful. The patient was a thin, emaciated bedouin. Serial blood pressure readings in the hospital were recorded between 90/60 and 220/120 mm Hg. During hypertensive crises, he developed a consistent pattern of chest pain, dyspnea, sweating, and sinus tachycardia up to 160 beats per minute. He had a firm goiter with a retrosternal extension and hepatomegaly (7 cm below the right costal margin). The rest of the physical examination was unremarkable.Laboratory investigations yielded the following results: hemoglobin, 115 g/L (normocytic normochromic anemia); leukocyte count, 5.7 10 9 /L; and erythrocyte sedimentation rate, 20 mm/h. Results of chemical analyses of the blood were within normal limits, except for fasting blood glucose level of 8.5 mmol/L. Levels of serum calcium, phosphorus, and alkaline phosphatase were all within normal limits on multiple determinations over a 2-year period.Results of serum hormone tests were as follows: thyroxine, 137.7 nmol/L (normal, 68 to 159.6 nmol/L); free thyroxine index, 41.31 nmol (normal, 16 to 38 nmol); calcitonin, 8300 and 5350 ng/L (normal, 0 to 28.5 ng/L); and carcinoembryonic antigen (CEA), 2450 and 960 nmol/L (normal, 0 to 500 nmol/L). Plasma norepinephrine levels were 33.1 and 4.86 nmol/L (normal, 0.6 to 4.1 nmol/L), and plasma epinephrine, 180 and 315 pmol/L (normal, 0 to 545.8 pmol/L).
Diagnostic ImagingA CT scan of the abdomen with contrast enhancement revealed a 12-cm, irregular, low-density mass in the right adrenal gland, poorly delineated from the liver, approaching the right hemidiaphragm, and with an incomplete rim of dense calcification. In the left adrenal gland, a 7-cm, irregular, low-density mass was seen. A slightly low-density mass in the right lobe of the liver measuring 7×3×3 cm was noted caudally and laterally consistent with a metastasis (Figure 1).An abdominal aortogram (Figure 2), with selective injections in the renal arteries (not shown), done at the time of attempted therapeutic embolization of the tumor revealed a vascular mass in the right adrenal bed fed by the right phrenic artery, right middle adrenal arteries, branches of the right renal artery, and from the lumbar arteries. A vascular mass in the left adr...