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CASE REPORT |
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| Year : 2000 | Volume
: 17
| Issue : 1 | Page : 52-53 |
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Cushing's syndrome in a patient with ACTH secreting pheochromocytoma
Dharm Raj Singh, K Gaitonde, SN Kulkarni, SN Sagade, NF Shah, A Bhaduri
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
India
 Source of Support: None, Conflict of Interest: None  | Check |
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 2020 Oct 30];17:52-3. Available from: https://www.indianjurol.com/text.asp?2000/17/1/52/41018 |
 Case Report | |  |
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 | | |
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 | | |
| 1. | Chen H, Doppman JL, Chrousos GP et al. Adrenocorticotropic hormone secreting pheochromocytoma: the exception to the rule. Surgery 1995; 118: 988-995.  |
| 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. |
| 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. |
[Figure 1], [Figure 2]
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