Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2009) 19 P56

SFEBES2009 Poster Presentations Clinical practice/governance and case reports (87 abstracts)

Pituitary ACTH-dependent Cushing’s syndrome caused by ectopic CRF secretion from an intra-adrenal phaeochromocytoma

D Woods 1 , V Arutchealvam 1 , A Madathil 1 , R Peaston 2 , S Turner 2 , RDG Neely 2 , J Graham 3 , D Richardson 3 , TJW Lennard 4 , R Bliss 4 , SB Bennett 5 , S Johnson 6 , JI Mason 7 & R Quinton 1

1Endocrine unit, Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK; 2Department Of Clinical Biochemistry, Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK; 3Department of Radiology, Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK; 4Department of Surgery, Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK; 5Northumbria Healthcare NHS Foundation Trust, Northumberland, UK; 6Department of Pathology, Newcastle Hospitals NHS Foundation Trust, Newcastle Upon Tyne, UK; 7Centre for Reproductive biology, Queen’s Medical Research Institute, Edinburgh, UK.

A 69-year-old woman was admitted with new uncontrolled diabetes, severe hypertension and bilateral leg oedema. Cushing’s syndrome was suspected when she developed resistant hypokalemic alkalosis on diuretic therapy. Detailed questioning also revealed a history of episodic sweating, palpitation and tremor.

Investigation & management: ACTH-dependant Cushing’s syndrome was confirmed: Serum ACTH 516 ng/l; urine free cortisol 20 045 nmol/24 h; 09.00am serum cortisol >2000 nmol/l post overnight dexamethasone test.

Phaeochromocytoma was also confirmed: normetanephrines 2590 umol/24 h (NR <1000) and 10.4 pmol/l (NR <3.8) in urine and plasma, respectively; metanephrines 330 umol/24 h (NR <600) and 4.0 pmol/l (NR <3.8) in urine and plasma, respectively; serum chromogranin-A 300 U/l (NR <30). MRI showed a hyper-intense 3 cm right adrenal mass, though MIBG and octeoscans were both negative. Pituitary MRI showed a possible right sided microadenoma, but high-resolution thoracic CT scan failed to reveal any significant lesion.

Her clinical and biochemical response to adrenal blockade was gratifyingly swift and complete (urine cortisol <50 nmol/24 h within a week) and she was maintained thereafter on Metyropone 0.5 mg/qds and dexamethasone 0.5 mg/bd.

BIPPS with CRH test

ACTH (ng/l)
TimeInferior petrosal sinusPeripheralInferior vena cava
243971620nb. Rt adrenal vein could not be cannulated

Discussion: We had initially postulated ectopic ACTH secretion from the phaeochromocytoma, but BIPPS unequivocally indicated that the source of ACTH was pituitary and not adrenal. The differential diagnosis there for lay between separate adrenal and pituitary pathologies and an adrenal lesion ectopically secreting CRF. A laparoscopic right adrenalectomy, preceded by incremental alpha-blockade, showed an intra-adrenal phaeochromocytoma. Post-operatively she had clinical and biochemical cure (off block-and-replace) of both endocrinopathies.

Conclusion: Ectopic hormone secretion from intra-adrenal phaeochromocytoma. is an extremely rare cause of Cushing’s syndrome. Of the few reported cases in the literature, most have been reported to secrete ACTH, rather than CRF.

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