Cushing's syndrome associated with an islet-cell tumour of the pancreas in a boy aged 2 years.

Abstract

We present this case because, so far as we can discover, no similar one has yet been reported in a child.Case History This boy was born in June 1963. He presented at the out-patient clinic in September of 1965 at the age of 2 years and 3 months, on account of sudden excessive weight gain. He had been healthy and free of symptoms until 6 weeks before, when he had suddenly developed a ravenous appetite ('he will eat two of every meal I offer him'). He had become very fat and his face had become very red. His weight four weeks before his attendance had been 12 7 kg.: now he weighed 15-9 kg. There were no other symptoms, and he was lively and cheerful, with no polydipsia or polyuria. He had never had a fit, faint, or episode of disordered consciousness, such as might be caused by hypoglycaemia. His birthweight had been 2-7 kg., and the perinatal period and infancy had been uneventful. The parents were both alive and well, and not of abnormal size or weight. The paternal great grandfather was short and fat. He had one brother aged 4 years alive and well, weighing at present 13-5 kg.Examination. He was very fat (Fig. 1), weight 15 9 kg. (97th centile), height 88 cm. (50th centile). He had a red moon face, and there was a faint brownish pigmentation of the entire skin, but no pigment inside the mouth. The genitalia were not enlarged and there was no hirsutes. Blood pressure estimations were difficult owing to the excessive amount of fat and to the child's dislike of the procedure. Two readings of 110 mm. Hg were recorded by the flush and palpation methods using a 10 cm. cuff. A firm, non-tender rounded mass about the size of an orange could be felt in the left hypochondrium. It did not move on respiration and did not extend backward into the loin. No other abnormalities were found. A provisional diagnosis was made of a rapidly growing, probably suprarenal tumour, and the child was admitted at once into the ward.Investigations. Table I

Adrenocorticotropin-Secreting Pancreatoblastoma

“…Doppman et al found only one adult (out of 53 cases) with islet cell tumor producing ACTH that was due to a benign adenoma 9 . The children reported all succumbed to their disease 1 " 9 , except the patient of Burkinshaw et al 6 who was tumor free at 1 year of follow-up. In their series of 12 patients, Amikara et al reported median survival of 2.5 years, with all patients showing evidence of recurrent disease 12 .…”