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WHO Classification of Tumours
ACTH producing adenoma
Pituitary gland


Definition

The ACTH producing adenoma is a benign tumour, derived from corticotrophs of the anterior pituitary, that synthesize proopiomelanocortin(POMC) from which several peptides including ACTH, β-LPH and β-endorphin are cleaved.

Pituitary ACTH-dependent disease is called Cushing disease as described by Harvey Cushing in 1932; this is in contrast to Cushing syndrome, which may have various nonpituitary aetiologies. Cushing disease is due to an ACTH producing adenoma, but the possibility of hyperplasia of corticotrophs, alone or associated with microadenoma, remains unclear

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Robert F, Hardy J (1991)
Cushing's disease: a correlation of radiological, surgical and pathological findings with therapeutic results.
Pathol Res Pract 187: 617-21




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Trouillas J, Guigard MP, Fonlupt P, Souchier C, Girod C (1996)
Mapping of corticotropic cells in the normal human pituitary.
J Histochem Cytochem 44: 473-9




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Young WF, Scheithauer BW, Gharib H, Laws ER, Carpenter PC (1988)
Cushing's syndrome due to primary multinodular corticotrope hyperplasia.
Mayo Clin Proc 63: 256-62



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ACTH producing adenomas with Cushing disease represent 10-15% of pituitary tumours

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Trouillas J (2002)
[Pathology and pathogenesis of pituitary corticotroph adenoma].
Neurochirurgie 48: 149-62



. They are rare in childhood
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Leinung MC, Kane LA, Scheithauer BW, Carpenter PC, Laws ER, Zimmerman D (1995)
Long term follow-up of transsphenoidal surgery for the treatment of Cushing's disease in childhood.
J Clin Endocrinol Metab 80: 2475-9



and the peak incidence is between the ages of 30 and 40 years. ACTH producing adenomas have an 8:1 female to male ratio
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Arafah BM, Nasrallah MP (2001)
Pituitary tumors: pathophysiology, clinical manifestations and management.
Endocr Relat Cancer 8: 287-305



.
The prognosis of ACTH producing adenomas is not related to the tumoural evolution but to the cardiovascular complications of the hypercorticism and to the neurosurgical difficulty in removing a very small tumour without damaging the juxtatumoural pituitary.