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Long-Term Survival with ACTH-Secreting Carcinoma of the Pituitary: A Case Report and Review of the Literature

Departments of Medicine (R.E.L., S.L.W.) and Radiology (A.G.K.), Columbia University College of Physicians and Surgeons, New York, New York 10032; Department of Medicine (M.H.), Milton S. Hershey Medical Center, Hershey, Pennsylvania 17033; and Emeritus Department of Medicine (R.E.P.), The New York Hospital–Cornell Medical Center, New York, New York 10021

Address all correspondence and requests for reprints to: Dr. Sharon L. Wardlaw, Department of Medicine, Columbia University College of Physicians and Surgeons, 630 West 168th Street, New York, New York 10032. E-mail: . sw22{at}columbia.edu

Abstract

A 48-yr-old woman was evaluated 21 yr after receiving treatment for an ACTH-secreting metastatic pituitary carcinoma. She had been diagnosed with Cushing’s disease 35 yr earlier at the age of 14 yr and had undergone bilateral adrenalectomy. Six years later she developed Nelson’s syndrome, which was treated with resection of a pituitary adenoma followed by radiotherapy to the sella turcica. Eight years later she was found to have craniospinal metastases with three remote intracerebral lesions. Two of these lesions were surgically resected and stained positive for ACTH by immunofluorescence. She subsequently received whole-brain radiotherapy and is doing well 21 yr later with no lesions seen on magnetic resonance imaging and no evidence of recurrent metastatic disease. We present this case in detail along with a literature review of ACTH-secreting pituitary carcinoma.

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