Abstract
ACTH-producing brain tumors in infancy are rare. An 8 month male was evaluated for a 4 month history of profound obesity, acne, moon-facies, hypertension, and hirsutism. An IVP and computerized tomographic (CT) scan of the abdomen were normal. Plasma DHEA was 155-299 ng/dl. Dexamethasone (3.75 mg/m2 × 3d) failed to suppress elevated urine 17-OHCS (10.1 mg/24h) and free cortisol (1026 μg/24h), but partially suppressed plasma cortisol from 79 to 32.4 μg/dl and plasma ACTH from 320 to 90 pg/ml. In response to TRF, TSH rose to 1.9 μU/ml, prolactin rose to 8.3 ng/ml and ACTH did not change. Brain CT scan and angiography demonstrated a large subfrontal tumor which appeared to be separate from the pituitary fossa. Craniotomy demonstrated an inoperable highly vascular tumor extending into the sella. Biopsy of the main body of the tumor showed undifferentiated mesenchymal tissue with some gland formation, while tumor superior to the sella histologically resembled pituitary adenoma. Medium from cultured cells from the mesenchymal tissue contained no measurable ACTH while medium from the cultured adenomatous cells contained 111-140 pg ACTH/ml. There was no bioassayable CRF activity in the medium from any cultured cells (assayed by Dr. Monte Greer).
Total adrenal weight at autopsy was 15 gm. The brain tumor was a 5×5×6cm mass with a tail extending into pituitary tissue. Serial histologic sections of the mass showed a gradual transition from pituitary adenoma with occasional gland-like structures, to sheets of undifferentiated mesenchymal tissue with venous sinusoids and numerous gland-like structures.
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Miller, W., Grumbach, M. & Kaplan, S. 315 ACTH-PRODUCING INTRACRANIAL TUMOR IN AN INFANT. Pediatr Res 12 (Suppl 4), 416 (1978). https://doi.org/10.1203/00006450-197804001-00320
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DOI: https://doi.org/10.1203/00006450-197804001-00320