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1/29. Intradural metastatic spinal cord compression from ACTH-secreting pituitary carcinoma.

    Pituitary carcinomas are uncommon and intradural metastatic spread to the spine is rarer still. We describe a 27-year old man with metastatic spinal cord compression from an adrenocorticotrophic hormone (ACTH) cell pituitary carcinoma, 16 years following the initial presentation. He had three previous resections of the pituitary tumour and post-operative radiotherapy. The intradural, extramedullary spinal metastases causing thoracic and lumbar cord compression were excised, with neurological improvement. Spinal metastases in pituitary carcinoma are uncommon, but aggressive surgical resection of the spinal metastases produces good symptomatic relief. ( info)

2/29. Long-term results of gamma knife surgery for growth hormone-producing pituitary adenoma: is the disease difficult to cure?

    OBJECT: The authors conducted a study to determine the long-term results of gamma knife surgery for residual or recurrent growth hormine (GH)-producing pituitary adenomas and to compare the results with those after treatment of other pituitary adenomas. methods: The series consisted of 67 patients. The mean tumor diameter was 19.2 mm and volume was 5.4 cm3. The mean maximum dose was 35.3 Gy and the mean margin dose was 18.9 Gy. The mean follow-up duration was 63.3 months (range 13-142 months). The tumor resolution rate was 2%, the response rate 68.3%, and the control rate 100%. growth hormone normalization (GH < 1.0 ng/ml) was found in 4.8%, nearly normal (< 2.0 ng/ml) in 11.9%, significantly decreased (< 5.0 ng/ml) in 23.8%, decreased in 21.4%, unchanged in 21.4%, and increased in 16.7%. serum insulin-like growth factor (IGF)-1 was significantly decreased (IGF-1 < 400 ng/ml) in 40.7%, decreased in 29.6%, unchanged in 18.5%, and increased in 11.1%, which was almost parallel to the GH changes. CONCLUSIONS: Gamma knife surgery was effective and safe for the control of tumors; however, normalization of GH and IGF-1 secretion was difficult to achieve in cases with large tumors and low-dose radiation. Gamma knife radiosurgery is thus indicated for small tumors after surgery or medication therapy when a relatively high-dose radiation is required. ( info)

3/29. Macronodular adrenocortical hyperplasia in a postmenopausal woman.

    This case report describes the diagnosis of Cushing's syndrome due to macronodular adrenal hyperplasia in an elderly woman who presented with fatigue, muscle weakness and oedema, and recent excessive bruising. Long-standing disease and comorbidity precluded adrenalectomy. Despite treatment with metyrapone and diuretics, the patient died after two months hospitalisation. Postmortal examination revealed overexpression of luteinising hormone (LH) receptors in the adrenal glands, suggesting that the postmenopausal rise in LH may have a role in adrenal hyperplasia and hypercortisolism. ( info)

4/29. Clinical manifestations and hormonal profile of two women with Cushing's disease and mild deficiency of 21-hydroxylase.

    The development of Cushing's disease among patients with deficiency of 21-hydroxylase has not been observed to date. The clinical manifestations and the hormonal profile of this exceptional association are herein described through the study of two cases. The first one was a 39-yr-old woman who had undergone non-curative transsphenoidal surgery for a pituitary-dependent Cushing's syndrome 12 yr before. She showed hypertension, central obesity, severe hirsutism, alopecia and hyperpigmentation. Urinary excretion of cortisol was normal, but ACTH levels were very high and hormonal dynamic studies (cortisol circadian rhythm, insulin-induced hypoglycemia and dexamethasone suppression tests) revealed the qualitative disturbances that characterize Cushing's disease. serum concentrations of androstenedione, free testosterone and 17-hydroxyprogesterone were clearly increased. Reexamination of the tissue samples from previous surgery confirmed the presence of an ACTH-producing pituitary adenoma. CYP21 gene analysis found the splicing 655G mutation at intron 2 and the V281L mutation at exon 7. The second case was a 21-yr-old woman who was diagnosed with pituitary ACTH-dependent Cushing's syndrome according to unequivocal clinical and laboratory findings. However, hirsutism was particularly severe and both serum androgens and 17-hydroxyprogesterone were elevated. The patient was heterozygote for a large conversion of CYP21 gene. In these cases, the clinical and biochemical expression of Cushing's syndrome was determined by the different severity of 21-hydroxylase deficiency and the subsequent residual ability of adrenal cortex to synthesize cortisol. ( info)

5/29. Effect of cabergoline treatment on Cushing's disease caused by aberrant adrenocorticotropin-secreting macroadenoma.

    The present case involves a 47-yr-old woman with Cushing's disease due to pituitary macroadenoma. The patient had suffered from hypertension and obesity for two yr. Her serum cortisol levels were moderately elevated throughout the observation period, and dexamethasone failed to suppress the cortisol secretion. plasma ACTH levels were markedly high (>100 pg/ml) and did not respond to CRH provocation. Gel filtration analysis of the patient's plasma detected the existence of big ACTH molecules, which eluted with a peak of authentic 1-39 ACTH. Cranial magnetic resonance imaging (MRI) revealed a 3 cm pituitary tumor occupying the sellar region and right cavernous sinus with diffuse enhancement by gadolinium. The pituitary mass was removed by transsphenoidal surgery, and was pathologically identified as compatible to ACTH-producing pituitary adenoma by immunohistochemistry. RT-PCR analysis of total cellular rna extracted from the resected adenoma revealed a relatively high expression level of dopamine D2 receptor (D2R) mRNA. Therefore, a long-acting D2R agonist, cabergoline (0.25 to 0.5 mg/week), was administered for the remnant adenoma, which gradually reduced ACTH levels in 90 days. In addition, cranial MRI exhibited shrinkage of the remnant pituitary mass after a 6-month treatment with cabergoline. This case demonstrates the efficacy of cabergoline to treat Cushing's disease caused by pituitary macroadenoma secreting aberrant ACTH molecules. ( info)

6/29. Ectopic adrenocorticotropic hormone hypersecretion due to a primary pulmonary paraganglioma.

    OBJECTIVE: To present a rare case of primary paraganglioma of the lung, which caused the syndrome of ectopic adrenocorticotropic hormone (ACTH) hypersecretion. methods: The clinical, biochemical, and imaging findings in this case are described, and the pathologic features of the resected tumor tissue are depicted. In addition, the related literature on paragangliomas is reviewed. RESULTS: In a 39-year-old woman with hypertension, weight gain, and easy bruising, laboratory studies showed hypercortisolemia, high plasma ACTH levels, and suppression of cortisol by high-dose dexamethasone. An indium-labeled octreotide whole-body scan disclosed a tumor in the left lower lung field. Thoracoscopic wedge resection of the pulmonary mass was performed. Postoperative microscopic findings and immunohistochemical stains revealed nests of rounded and polyhedral cells and S-100 protein-positive sustentacular (supporting) cells, charac-CONCLUSION: This unusual case of Cushing's syndrome was attributable to an ACTH-secreting primary pulmonary paraganglioma. ( info)

7/29. pregnancy in Cushing's disease shortly after treatment by gamma-knife radiosurgery.

    Gamma-knife radiosurgery (GKR) is considered as a possible treatment for patients affected by unsuccessfully surgically treated pituitary adenoma or not suitable for surgery. The disadvantages of this technique seem to be the length of time to the onset of remission, which is known to be at least of 6 months, and the possible adverse effects. We report here a case of a 13-yr-old female patient with Cushing's disease (CD) due to acth-secreting pituitary adenoma. After a complete clinical remission obtained by a transsphenoidal surgery, at the age of 18, the patient had a recurrence of ACTH-dependent hypercortisolism, and a second transsphenoidal surgery was performed. In April 1999, a second recurrence of CD was diagnosed and the patient underwent GKR on a small pituitary mass, on the left side of the sella. In June 1999 amenorrhoea appeared, and in August 1999 pregnancy occurred. Although during the pregnancy the disease activity was still high, the fetus's growth was normal and in February 2000 a normal male infant was delivered. The baby and the mother did not show any biochemical signs or clinical symptoms of hypo- or hypercortisolism. This case is interesting, since GKR exerted a very rapid effect and turned to be safe even if performed shortly before pregnancy. Moreover, in spite of the still high disease activity, the pregnancy had a normal course and the fetus did not have any cortisol secretion abnormalities. ( info)

8/29. craniopharyngioma and Cushing disease: case report.

    craniopharyngioma is a common sellar region tumor occurring in children. It usually manifests as endocrinological deficits such as short stature, delayed puberty, and obesity. patients with craniopharyngioma commonly present with visual deficits and hydrocephalus. The authors present the case of a child who presented with short stature and clinical evidence of Cushing disease (CD) associated with a suprasellar tumor. The patient underwent insertion of an Ommaya reservoir into the tumor's cystic portion. High adrenocorticotropic hormone (ACTH) levels were demonstrated within the cyst's fluid and in the serum. After adequate decompression of the tumor, the patient underwent total resection. The tumor pathology was compatible with an adamantinomatous craniopharyngioma and immunohistochemical studies failed to show staining for ACTH. Panhypopituitarism developed postoperatively in the patient and he received hormone substitution therapy with final adequate height and normal-high weight. The neurosurgical implications of CD along with a possible mechanism for this patient's presentation are discussed in detail on the basis of the pertinent literature. ( info)

9/29. Periodic secretion of adrenocorticotropin in a patient with Cushing's disease manifested during pregnancy.

    We report the case of 19-year-old woman with cyclical Cushing's disease, in whom plasma adrenocorticotropin (ACTH) was secreted periodically after her first pregnancy. Since the 33rd week of pregnancy, hypertension and proteinuria became clinically remarkable. She gave normal birth at 36th week of pregnancy; however she continued to gain body weight even after delivery and developed typical Cushingoid features. Her ACTH secretion lacked normal daily fluctuation but exhibited periodic change during 1-year observation, showing 119 pg/ml, 34.6 pg/ml and 115 pg/ml at the 4th, 7th and 13th months after delivery. plasma ACTH levels were increased by corticotropin releasing hormone and metyrapone, while low-dose dexamethasone suppressed cortisol secretion. Gel filtration analysis of the patient's plasma detected big ACTH molecules being eluted with a peak of authentic 1-39 ACTH. Cranial magnetic resonance imaging revealed a 1-cm pituitary mass in right cavernous sinus. The pituitary tumor was removed by transsphenoidal surgery at 13th month after delivery and was pathologically compatible with ACTH-producing pituitary adenoma by immunohistochemistry. This case includes clinically rare subsets of Cushing's syndrome showing periodic ACTH secretion and aberrant ACTH molecules. ( info)

10/29. pseudotumor cerebri in patients with Cushing's disease.

    OBJECTIVE: To discuss the manifestation of pseudotumor cerebri during the course of correcting the hypercortisolism of Cushing's disease. methods: We describe the clinical, biochemical, and neuro-ophthalmologic findings in a patient in whom pseudotumor cerebri developed during correction of the hypercortisolism of Cushing's disease. In addition, we review the relationship between pseudotumor cerebri and Cushing's disease in the literature. RESULTS: A 44-year-old woman with Cushing's disease underwent total adrenalectomy after two unsuccessful transsphenoidal operations. She developed daily headaches 2 weeks postoperatively while taking 60 mg of hydrocortisone daily. She noticed a visual floater 8 weeks postoperatively, and a prompt ophthalmologic evaluation revealed papilledema. We diagnosed pseudotumor cerebri on the basis of symptoms and signs of intracranial hypertension, unremarkable findings on neuroimaging and cerebrospinal fluid analysis, and a substantially increased cerebrospinal fluid pressure. After 8 weeks of treatment with furosemide, the headaches and papilledema resolved, and the patient was able to reduce her dose of hydrocortisone to 30 mg daily. pseudotumor cerebri has been associated with adrenocortical insufficiency and gluco- corticoid withdrawal and can lead to loss of vision. A literature review revealed 6 previous patients in whom pseudotumor cerebri developed in association with Cushing's disease. In all but one case, the pseudotumor cerebri manifested 2 to 4 weeks after treatment of hypercortisolism or cessation of hydrocortisone replacement. CONCLUSION: Symptoms of intracranial hypertension such as headache occurring in a patient recently withdrawn from exogenous or endogenous corticosteroids should prompt consideration of the presence of pseudotumor cerebri. ( info)
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