An acth-secreting pituitary tumour arising in a patient with congenital adrenal hyperplasia.

Abstract:

:The case reported is of a 46-year-old woman who had congenital adrenal hyperplasia due to a 21-hydroxylase deficiency, and in whom there was the development of an ACTH secreting pituitary tumour. The patient was untreated with glucocorticoids until the age of 32 years when she presented with infertility. She next presented with amenorrhoea at the age of 44 years when she was found to have an enlarged pituitary fossa. Despite treatment with bromocriptine and adequate doses of dexamethasone, the tumour enlarged and required operative treatment 1 year later. Before and after operation, plasma ACTH levels were between 300 and 400 ng/l, immunocytochemistry showed staining for ACTH and other structurally related pro-opiocortin peptides but for no other hormones, and the tumour secreted large amounts of ACTH in vitro. The report of this case is to our knowledge the first account of a feedback tumour in congenital adrenal hyperplasia and provides yet another reason why patients with this condition should be treated, and good control achieved.

journal_name

Clin Endocrinol (Oxf)

journal_title

Clinical endocrinology

authors

Horrocks PM,Franks S,Hockley AD,Rolfe EB,Van Noorden S,London DR

doi

10.1111/j.1365-2265.1982.tb01613.x

subject

Has Abstract

pub_date

1982-11-01 00:00:00

pages

457-68

issue

5

eissn

0300-0664

issn

1365-2265

journal_volume

17

pub_type

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