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Year : 2000  |  Volume : 17  |  Issue : 1  |  Page : 52-53

Cushing's syndrome in a patient with ACTH secreting pheochromocytoma

Departments of Urology, Endocrinology and Histopathology, RD. Hinduja National Hospital and Medical Research Centre, Mumbai, India

Correspondence Address:
S N Sagade
Consultant Urologist, P.D. Hinduja National Hospital and Medical Research Centre, V.S. Marg, Mahim, Mumbai - 400 016
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Source of Support: None, Conflict of Interest: None

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Keywords: Pheochromocytoma; Cushing′s Syndrome

How to cite this article:
Singh DR, Gaitonde K, Kulkarni S N, Sagade S N, Shah N F, Bhaduri A. Cushing's syndrome in a patient with ACTH secreting pheochromocytoma. Indian J Urol 2000;17:52-3

How to cite this URL:
Singh DR, Gaitonde K, Kulkarni S N, Sagade S N, Shah N F, Bhaduri A. Cushing's syndrome in a patient with ACTH secreting pheochromocytoma. Indian J Urol [serial online] 2000 [cited 2023 Feb 5];17:52-3. Available from:

   Case Report Top

A 37-year-old female presented with progressive weight gain, rounding of the face and backache since 5 months. She was normotensive and non-diabetic. Physical exami­nation revealed typical Cushingoid features, i.e., centri­petal obesity, mooning of face, buffalo hump, broad, livid and tapering striae on the abdomen, axilla, breast and thigh. Mild hirsutism and hyperpigmentation of knuckles were also present. On investigation the serum cortisol at 8 a.m. was 25 mcg/dl (n=8-24) at 4 p.m. was 19 mcg/dl (n=2­15). Post-dexamethasone (overnight, I mg), serum corti­sol was 29 mcg/dl (n<5) and post-dexamethasone (overnight, 8 mg) serum cortisol was 53.9 mcg/dl. 24 hours' urinary cortisol excretion was 1566.3 mcg (n=20­90). The serum ACTH (Adrenocorticotrophic hormone) value was 21.9 pg/ml (n=0-46). Computerized tomogra­phy (CT) scan of the abdomen revealed 5.2x4 cm mass in the right adrenal gland [Figure 1]. Right adrenelectomy was performed via 11 th rib flank incision. Postoperative recov­ery was uneventful. The serum cortisol on 5 th postop day was 10 mcg/dl. Histopathology and immunohistochemis­try revealed ACTH secreting pheochromocytoma [Figure 2].

   Comments Top

Ectopic (non-pituitary) secretion of ACTH is responsi­ble for Cushing's syndrome in 10-15% of patients. [1] The common sources for ectopic ACTH secretion are small­cell lung cancer, bronchial carcinoid tumours, pancreatic islet cell tumours, thymic carcinoid tumours and medul­lary thyroid cancer. [2] Meloni (1966) first described the ec­topic ACTH secreting pheochromocytoma. [3] Till 1995, 21 cases of ACTH secreting pheochromocytoma have been described in the world literature. [1] In the present case, clinical diagnosis of Cushing's syndrome was based on clini­cal features supported by the elevated basal serum corti­sol and 24 hours urinary cortisol. A non-suppressible post-dexamethasone serum cortisol with a normal serum ACTH led to suspicion of non-pituitary cause of Cush­ing's syndrome which was confirmed by the CT scan of the abdomen. Since pheochromocytoma was not suspected clinically, laboratory tests for its diaganosis were not per­formed. Although the normal value of serum ACTH ruled out the primary adrenal cause of Cushing's syndrome but in view of right adrenal mass in CT scan without contral­ateral adrenal hyperplasia, the ectopic ACTH production was not suspected pre-operatively. Diagnosis was made by the immunohistochemistry of the tumour which stained positive for ACTH along with other features of pheo­chromocytoma.

   References Top

1.Chen H, Doppman JL, Chrousos GP et al. Adrenocorticotropic hor­mone secreting pheochromocytoma: the exception to the rule. Sur­gery 1995; 118: 988-995.  Back to cited text no. 1    
2.Zeiger MA, Pass HI, Doppman JD et al. Surgical strategy in the management of non-small-cell ectopic adrenocorticotropic hormone syndrome. Surgery 1992; 112: 994-1001.  Back to cited text no. 2    
3.Bruining HA, On- EGL, Gershuny AR et al. Cushing's syndrome and pheochromocytoma caused by an adrenal tumour, also con­taining met-enkephalin and somatostatin: a case report. World J Surg 1985: 9: 639-642.  Back to cited text no. 3    


  [Figure 1], [Figure 2]


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