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Endocrinologic, biochemical and histologic characterization of an ectopic ACTH producing carcinoma in childhood

We studied a 10 yr old boy with Cushing's syndrome due to an ACTH secreting islet cell carcinoma of the pancreas. He had rapid weight gain, abdominal distention, proximal weakness but no hirsutism or striae. Height was -1 SD, weight was +2 SD and BP 130/90. Serum cortisol was 50-95 μg/dl without diurnal variation. Urinary free cortisol was 840 μg/24 hrs and not suppressed by dexamethasone. Plasma ACTH was strikingly elevated and secreted episodically at 900-1600 pg/ml with similarly elevated βLPH. Plasma calcitonin, LDH and α-fetoprotein concentrations were elevated. Hypokalemic alkalosis was present with normal plasma aldosterone but elevated renin and DOC. Plasma insulin to glucose ratio was normal. CT scan showed areas of punctate calcification in tail of pancreas. A tumor in the tail of the pancreas with extensive tumor nodules over the serosa was found. Tumor and both hyperplastic adrenals were removed to ameliorate Cushing's syndrome. An islet cell carcinoma was diagnosed histologically. Immunohistochemistry localized ACTH and insulin throughout the tumor. Tumor contained large amounts of BLPH by RIA. SDS gel electrophoresis of immunoprecipitated cell free translation products coded by tumor mRNA indicated that ACTH and BLPH arose from a common precursor molecule of 35000 daltons. A second patient, a 10 yr old girl with the same type of ACTH secreting islet cell CA was studied in 1957.