OBJECTIVE: To report a case of recurrent Cushing's disease after an apparent cure and long-term surveillance. METHODS: We describe in detail the follow-up course of a woman who underwent transsphenoidal resection of a corticotropin-secreting pituitary microadenoma in 1981. RESULTS: For 2 years postoperatively, the patient exhibited adrenocortical insufficiency. In 1983, the pituitary-adrenal axis was normal. She remained eucorticoid until May 1992, when features of Cushing's syndrome redeveloped. Plasma and urine cortisol and plasma corticotropin levels were once again increased in conjunction with a loss of diurnal variation and abnormal responses to dexamethasone suppression. A magnetic resonance imaging scan of the pituitary gland was consistent with a small lesion on the left side, and petrosal sinus sampling after ovine corticotropin-releasing hormone stimulation was consistent with increased activity on the right side. CONCLUSION: This 10-year interval between apparent cure and recurrence appears to be the longest thus far reported for a patient with Cushing's disease. These observations reinforce the impression that permanent cure of this disorder is uncertain and indicate the need for indefinite follow-up.

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PubMedDate not supplied

Intravenously administered pamidronate for treating refractory Paget's disease of bone.

OBJECTIVE: To report our experience with the use of intravenously administered pamidronate in 16 patients with refractory Paget's disease of bone. METHODS: We describe our treatment regimen and outline serum alkaline phosphatase levels at baseline and after pamidronate therapy in our study cohort. In addition, we summarize clinical symptoms and response to treatment. RESULTS: Although clinical experience with pamidronate in the treatment of Paget's disease of bone has been limited in the United States, elsewhere it has been shown to be an effective agent that inhibits the increased osteoclastic activity characteristic of this disease. Accordingly, in 16 patients with Paget's disease unresponsive to various other therapies, we administered pamidronate intravenously at a dose of 30 mg in 500 mL of 5% dextrose in water during a 4-hour period once weekly for 6 weeks. Serum alkaline phosphatase was determined at baseline and at regular intervals for at least 12 months after the onset of therapy. Many of these patients reported relief of pain and an increased flexibility or range of motion after treatment. The only adverse effect reported by these patients was an acute-phase reaction during the first infusion in two patients. In all patients, serum alkaline phosphatase levels declined, and significant (P = 0.0012) decreases from baseline were noted within 6 weeks after the initial infusion. Maximal responses were generally seen within 6 months after treatment, serum alkaline phosphatase levels decreasing as much as 91% from baseline. CONCLUSION: These data suggest that intravenous pamidronate therapy is an effective alternative to calcitonin and etidronate in the treatment of Paget's disease of bone, particularly in those patients refractory to such agents.

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PubMedDate not supplied

Nelson's syndrome due to an intracavernous corticotropin-secreting adenoma.

OBJECTIVE: To report the first case of Nelson's syndrome due to an ectopic intracranial corticotropin-secreting tumor arising entirely within the cavernous sinus. METHODS: We present a case report of Nelson's syndrome with clinical, laboratory, and radiologic features throughout a 25-year period. RESULTS: A 54-year-old woman had been treated for Cushing's disease with bilateral adrenalectomy in 1971. Subsequently, Nelson's syndrome developed, and she had severe generalized hyperpigmentation and substantially increased plasma corticotropin levels. In 1976, she underwent a transsphenoidal hypophysectomy. Postoperatively, despite the development of panhypopituitarism and diabetes insipidus, she remained hyperpigmented and had persistently increased plasma corticotropin levels. Throughout the years, efforts to identify the site of the corticotropin-secreting tumor were unsuccessful until 1988, when magnetic resonance imaging revealed a mass in the right cavernous sinus; subsequently, petrosal sinus cannulation corroborated the intracavernous source of excess corticotropin. Cobalt-60 gamma knife radiotherapy in 1992 was followed by a clinical and hormonal response 4 1/2 years later. CONCLUSION: This report describes only the second reported case of an intracranial corticotropin-secreting tumor arising entirely within the cavernous sinus and the first such case associated with Nelson's syndrome. Although rare, the possibility of an ectopic intracranial or extracranial pituitary adenoma should be considered in patients with pituitary hypersecretion without clear-cut intrasellar abnormalities or those with no response to surgical resection of the pituitary gland.

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