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Year : 2008  |  Volume : 12  |  Issue : 4  |  Page : 9-12

Adrenal Glands In Patients With Spontaneous Hyperocortisolism : A Retrospective Comparison Of Those Who Developed Nelson’s With Those Who Are Asymptomatic For At Least 6 Years Postsurgery



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M Ashraf Ganie


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Introduction : Although up to 90% of patients with Cushing’s disease go into remission following pituitary surgery, a substantial number relapses. Nodular hyperplasis of pituitary seen in some cases of Cushing’s disease is believed by some experts to be an indicator of primary hypothalamic defect and this party explains such high rate of disease recurrence. However nearly 50 % of all patients who undergo bilateral adrenalectomy as primary modality of treatment remain in remission without showing any evidence of pituitary or ectopic ACTH excess over long period of follow up. This raises the possibility of a primary adrenal pathology such as presence of aberrant ACTH- receptor in adrenal or adrenal stimulatory antibody. Objectives : To compare the histological features of adrenal glands in patients developing Nelson’s syndrome with those without any evidence of corticotroph adenoma or ectopic. ACTH excess after bilateral adrenalectomy. Material and Methods : Records of patients who underwent bilateral adrenalectomy as a primary modality of treatment after 1985 at our center and no regular follow up were reviewed. The patients were divided in to two groups I, Nelson’s syndrome and II, > 9 years of uneventful follow up. Histopathologic features of surgically removed adrenal were reviewed by an experienced pathologist who was blind to the background and outcome of patients. Focal lympho-plasmacytic infiltrate (FLPI) was intensely looked for and graded as mild, moderate and prominent. Results : There were a total of sixteen patients (5 M and 11 F) with a mean age of 27.6 ± 7.3 years (range of 14 – 42 year). Five patients (31.25%) developed Nelson’s syndrome and underwent pituitary surgery while eleven completed > 9 years (6-16; median 9 years) follow up without any evidence of pituitary or ectopic ACTH excess. All patients in either group had diffuse cortical hyperplasia. FLPI was seen in only two out of seven (28.57%) Nelson’s syndrome compared to five of eleven (45.45%) in the second group (16.6% mild, 66.6% moderate and 16.6% prominent). Conclusion : Absence of a pituitary adenoma at diagnosis and long postoperative follow up in some patients casts doubt over universality of primary role of pituitary in Cushing’s disease. The present analysis reveals much higher prevalence of lympho-plasmacytic infiltrate in addition to diffuse cortical hyperplasia in those who did not develop Nelson’s syndrome following bilateral adrenalectomy indicating possible role of autoimmune mechanism of hypercortisolism. Well designed prospective study including more patients is required to refute or confirm this possibility.


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