CUSHING'S SYNDROME is uncommon in childhood and rare in infancy. In these age groups it is usually due to adrenal cortical tumors, although cases of adrenal hyperplasia are recorded. Approximately 60 adults have been reported, in whom Cushing's syndrome developed in association with nonendocrine tumors, ie, tumors not of pituitary, adrenocortical, or gonadal origin. Our patient had an adrenal neuroblastoma and was similar to the majority of these adults, in that sudden onset of symptoms, marked weakness associated with hypokalemia, and a rapidly fatal outcome characterized his course. This is the second report of Cushing's syndrome in an infant in association with a neuroblastoma. It is possible that awareness of the salient features of these cases might permit earlier diagnosis, and possibly complete tumor removal.
MethodsA modification of the method of Callow et al was used to measure 17-ketosteroids,1 and 17\ x=req-\ hydroxycorticosteroids were measured by a modification of the method of Glenn and Nelson.2 Norepinephrine and epinephrine were measured by a modification of the method of Crout,3 and vanillyl mandelic acid (VMA) by the method of Armstrong et al.4 An attempt to estimate adrenocorticotrophic hormone (ACTH) activity in the serum and the tumor was undertaken by a modification of the meth¬ od of Williams et al.5
Report of a CaseThe patient was born Oct 29, 1959, and grew normally in height, but at 18 months began to gain weight rapidly. Over the next three months he gained 7 lbs (expected gain 1 lb from age 18 to 21 months). At age 20 months, one month prior to admission, he had sudden onset of lassitude: he would not go upstairs and preferred to be carried rather than to walk. His private physician examined him and found the blood pressure to be normal and no abnormali¬ ties were noted on physical examination. The urinalysis was reported as "normal." One month after this examination, he was found comatose in bed. The temperature was 104 F (40 C). He began "shaking all over" and was admitted to another hospital. There, an ab¬ dominal mass was palpated; the serum potas¬ sium was 2.1 and serum sodium 149 mEq/liter. While in a semicomatose condition, he was transferred later the same day to the Chil¬ dren's Hospital in Pittsburgh. At age 21 months, his height was 86 cm (2 ft 10 inches, height age 24 months) and weight 18 kg (39 lbs 11 oz, 50% overweight for height). The chest measured 58.5 cm, the circumference expected for a 7-year-old. The blood pressure was 175/125 (average for age is 85/60), pulse 160, and temperature 102.2 F (39 C). He had a typi¬ cal cushingoid appearance with facial hirsutism, but without striae or hyperpigmentation. Deep tendon reflexes were unobtainable. A large mass occupied the left upper quadrant of the distended abdomen. The Laboratory Studies.-Hemoglobin was 9 gm/100 cc; hematocrit, 30%; leukocytes, 13,-500/cu mm; with 92% polymorphonuclears; platelet count, 200,000/cu mm; two bone mar¬ row aspirations, normal; urinary pH, 7.5; sub¬ sequent urinary pH determinations were nev...
