Clinical characteristics of silent corticotrophic adenomas and creation of an internet-accessible database to facilitate their multi-institutional study.

OBJECTIVE: Silent corticotrophic adenomas (SCAs) of the pituitary gland present as clinically nonfunctioning sellar lesions, with normal serum and urine hormone testing results, but stain positively for adrenocorticotropic hormone in immunohistochemical analyses. These tumors are now more readily recognized, but determination of their natural history and responses to treatment is difficult because of their rarity. We report the diagnoses and outcomes for a series of patients with SCAs, and we describe the creation of an Internet-accessible database (www.hsc.virginia.edu/neuro/neurosurgery/pituitary.html) for collection of multi-institutional data on these lesions. METHODS: The medical records of patients with documented SCAs who were treated at the University of Virginia between 1991 and 2002 were reviewed. A comprehensive data collection form was then created and posted online. RESULTS: Twenty-seven patients with SCAs were identified, with a female predominance (70%, P = 0.04). Headache was the most common presenting symptom (70%), followed by visual field deficits (52%), acute or subacute pituitary apoplexy (33%), cavernous sinus syndrome (18.5%), and hypopituitarism (11.1%). Extrasellar extension was noted for 92.6% of patients on preoperative magnetic resonance imaging scans. Transsphenoidal surgery was performed for all patients. Follow-up information was available for all patients (median, 60 mo; range, 3-254 mo). Postoperatively, 33% of patients received radiotherapy. Recurrence was noted for 37% of all patients and 41.7% of patients who did not receive postoperative radiotherapy. CONCLUSION: SCAs, although clinically nonfunctioning, may behave like aggressive adrenocorticotropic hormone-secreting adenomas and therefore should receive vigorous follow-up monitoring, with consideration being given to the recommendation of radiotherapy in cases with residual tumor.

[Cushing's disease]. / Maladie de Cushing.

Patients with Cushing's disease usually present typical clinical and biological features easily leading to the diagnosis. However very atypical presentations of the disease do exist especially in the intermittent forms and several investigations are often necessary to detect pituitary microadenoma wich sometimes is inconspicuous. The course of the disease can be serious and might responsible of definitive sequellas wich can threaten patient's life. Therefore rapid treatment is recommended. Pituitary surgery should be considered as the major therapeutic approach; however, in severe cases or when pituitary-directed treatments or OP'DDD have failed, total bilateral adrenalectomy should be proposed.

Is magnetic resonance imaging more sensitive than computed tomography in the location of corticotropin secreting pituitary adenomas?

Two cases of pituitary-dependent Cushing's syndrome are described in which the computed tomography (CT) examination was negative; as the hormone dynamic investigations were directed towards the presence of corticotropin (ACTH) secreting pituitary formations, magnetic resonance imaging (MRI) of the pituitary was performed, which evidenced the presence of such lesions; subsequent neurosurgery confirmed in both cases the location indicated by MRI. In conclusion, the higher sensitivity of MRI compared to CT in the diagnosis of ACTH secreting pituitary adenomas can be noted.

Cushing disease presenting as atypical psychosis followed by sudden death.

The authors report a case of Cushing's Disease that first presented as a diagnostically confusing Atypical Psychosis. Sudden death occurred secondary to unexpected gastrointestinal bleed. Pathological findings confirmed the diagnosis. Neurochemical mechanisms for the behavioral aberrations are explored.

Diagnosis, treatment and postoperative results of Cushing's disease.

One hundred patients with Cushing's disease were operated on in Nagoya University Hospital between January 1977 and January 1984. Pituitary adenomas were found and resected in 93 cases and complete clinical remissions were observed in 89 treated by operation alone and in two additional cases by operation followed with radiotherapy. The clinical features, pre-operative endocrine data, radiological findings including high resolution CT with coronal and sagittal reconstructions, data of selective venous sampling, operative findings, tumour pathology, postoperative clinical course, postoperative endocrine results and follow-up findings are presented and discussed.

Mania in Cushing's syndrome: case report.

A case is described of recurrent mania without depressive episodes in a patient subsequently diagnosed as having an ACTH-producing basophil adenoma. The difficulties involved in the clinical and laboratory diagnosis of Cushing's syndrome, and the ramifications of neuroendocrinopathies' ability to mimic symptoms of functional psychosis, are discussed.

Postadrenalectomy pituitary adenoma (Nelson's syndrome) in childhood: clinical and roentgenologic detection.

Following total adrenalectomy in Cushing's syndrome associated with adrenal hyperplasia, but not with adrenal adenoma or malignancy, elements of the pituitary may be stimulated to grow rapidly and even aggressively. There is strong evidence to support the idea that there is a pre-existing tumor in many, if not all, of these cases. In some, the tumor may be too small to deform the sella turcica. After adrenalectomy, these patients should be observed carefully and frequently for hyperpigmentation, visual field defects, and sella turcica enlargement.