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Results for “Pituitary ACTH Hypersecretion”

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At least 19 recordsLinked to original sources
PubMed2005

Cyclic Cushing's disease with paradoxical response to dexamethasone.

Cyclic Cushing's disease is an unusual disorder characterised by ACTH-dependent periodical increase of serum cortisol levels, clinically accompanied by peripheral edema, abnormalities of cardiac rhythm and hypokalemia. The condition may be unrecognised for years, since the typical features of Cushing's disease are usually absent due to the intermittent and brief duration of cortisol hypersecretion. We describe the case of a 42-yr-old man with Cyclic Cushing's disease due to an ACTH-producing pituitary macroadenoma, who presented two episodes of hypercortisolism in a 3-yr-period, clinically characterised by peripheral edema, hypokalemia and arrhythmia. The diagnosis was suspected because of a paradoxical increase of plasma ACTH and cortisol after dexamethasone administration during an asymptomatic period and was confirmed by pituitary imaging and by final histology after transphenoidal resection of the pituitary adenoma. After surgery, the patient resumed a normal pituitary-adrenal function with restoration of the normal ACTH and cortisol suppression after dexamethasone. Cyclic Cushing's disease should be considered in the differential diagnosis of several conditions characterised by recurrent episodes of idiopathic edema, hypokalemia or unexplained cardiac arrhythmia. In such patients, the pituitary-adrenal axis should be tested possibly during the acute phase of their disease or using the dexamethasone suppression test during asymptomatic intervals.

Adenoma↗
PubMed1988

Hypercortisolism due to an autonomously functioning pituitary adenoma: limitations in diagnostic test procedures.

We report a patient with pituitary-dependent Cushing's syndrome in whom standard biochemical testing procedures failed to define the pituitary as the source of autonomous hormone secretion. Anomalous findings included: 1) wide variations of urinary steroid excretion during a 4-day dexamethasone suppression test, 2) a rise of the serum cortisol after the single dose (8 mg) overnight dexamethasone suppression test, 3) a diminished serum 11-deoxycortisol response to the metyrapone test, and 4) an absent plasma ACTH response to CRH. While the results of these studies were most consistent with an ectopic source of ACTH production, magnetic resonance imaging suggested that Cushing's syndrome was due to a pituitary adenoma. The adenoma was removed by transphenoidal surgery. However, serum and urinary steroid levels remained measurable for 2 weeks because of the perioperative use of im cortisone acetate. Subsequent im administration of 200 mg cortisone acetate again led to prolonged serum and urinary steroid responses to this compound, thus emphasizing the pitfalls that may accompany the perioperative use of cortisone acetate. This patient demonstrates the importance of performing multiple tests, including magnetic resonance image scanning, in evaluation Cushing's syndrome, since standard biochemical testing may not indicate pituitary ACTH hypersecretion as the cause.

Adenoma↗
PubMed1987

Cushing's disease.

Cushing's disease is a form of Cushing's syndrome, persistent inappropriate hypercortisolism, that results from pituitary ACTH hypersecretion. It currently accounts for 70 per cent of adult cases of Cushing's syndrome and affects mainly women of childbearing age. The pathology, cause, and clinical and laboratory features of the disease are discussed. The initial step in diagnosis is documentation of endogenous hypercortisolism, which is followed by identification of the cause. Selective transsphenoidal resection of ACTH-producing pituitary adenomas is the initial treatment of choice. The roles of radiotherapy and medical therapy are discussed.

Adrenalectomy↗
PubMed2005

Repeated transsphenoidal surgery to treat recurrent or residual pituitary adenoma.

OBJECT: In this paper the authors describe the indications for and the results and complications of repeated transsphenoidal surgery (RTSS) to treat recurrent or residual pituitary adenoma. METHODS: A retrospective review was conducted of 96 consecutive patients who underwent RTSS to treat recurrent or residual pituitary adenoma. Ninety-six patients underwent RTSS: 42 to treat a recurrent or residual pituitary mass and 54 to treat a recurrent or persistent hormone hypersecretion. There was no case of perioperative death and there was a 1% incidence of major complications. Postoperative endocrinological deficiencies were uncommon unless planned total hypophysectomy was performed to treat Cushing disease. Clinical remission occurred in 93% of patients undergoing RTSS to treat a tumor mass, and 15% of patients initially experienced remission only to face a relapse after a mean of 32 months. Endocrinological remission occurred in 57% of patients undergoing RTSS to treat hormone hypersecretion; most of these patients had Cushing disease. Thirty-five percent of patients with an initial endocrinological remission experienced a relapse of their symptoms after a mean of 31 months (thus, 37% of patients achieved sustained endocrinological remission). We failed to identify factors that accurately predicted initial symptom remission or delayed relapse following RTSS. Ten patients in our series eventually underwent a third transsphenoidal surgery without major complications. CONCLUSIONS: Repeated transsphenoidal surgery is a more effective treatment for recurrent or residual mass than it is for hormone hypersecretion and has acceptable rates of morbidity and mortality. If hypophysectomy is not performed, endocrinological deficiencies are unlikely following RTSS.

Adenoma↗
PubMed2004

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.

Adenoma↗
PubMed2005

Pituitary-adrenal dynamics after ACTH-secreting pituitary tumor resection in patients receiving no steroids post-operatively.

It has recently been suggested that the classical routine of glucocorticoid administration before and after transsphenoidal surgery (TSS) in Cushing's disease (CD) patients may not be necessary, since it is likely that peritumoral normal corticotrophs are not completely suppressed during this period. We compared the dynamics of ACTH and cortisol from a group of CD patients (cured and not cured), receiving no steroids post-operatively, with a control group of acromegalic patients who presented normal hypothalamic-pituitary-adrenal (HPA) axis. Blood samples for ACTH and cortisol determination were obtained immediately before, at the end of surgery and at 4, 8, 12, 16, 24, 48 and 72 h after surgery, in 8 cured CD patients (Group I), 9 not cured CD patients (Group II) and in 7 subjects with acromegaly (Group III) who presented normal HPA axis (control group). The mean ACTH level in Group I was significantly lower than in Group III from 4 to 12 h and lower than in Group II from 8 to 12 h post-operatively. The mean cortisol level in Group I was lower than in Groups II and III from 8 to 72 h after surgery. No difference in mean cortisol level was observed among Groups II and III during the evaluated period. The lowest cortisol value in Group II was 193 nmol/l (at 24 h after surgery) and in Group I patients, after 20 h post-operatively, the highest cortisol level was 165 nmol/l. Although all cured CD patients (Group I) presented serum cortisol level lower than 55 nmol/l until 72 h after surgery, none had significant complications related to adrenal insufficiency. Ours findings are in agreement with recent observations that there is probably no need for glucocorticoid administration until clinical and/or laboratorial data are suggestive of adrenal insufficiency. However, we have also shown that a subphysiological HPA axis response could be observed in cured CD patients after TSS, and a definitive conclusion about glucocorticoid management during and after this procedure could not be made on the ground of the few cases studied in the literature.

Adenoma↗
PubMed2004

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.

Adenoma↗
PubMed2004

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.

Adenoma↗
PubMed2004

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.

Carcinoma↗
PubMed2004

The Nelson's syndrome... revisited.

Adrenalectomy is a radical therapeutic approach to control hypercortisolism in some patients with Cushing's disease. However it may be complicated by the Nelson's syndrome, defined by the association of a pituitary macroadenoma and high ACTH secretion after adrenalectomy. This definition has not changed since the end of the fifties. Today the Nelson's syndrome must be revisited with new to criteria using more sensitive diagnostic tools, especially the pituitary magnetic resonance imaging. In this paper we will review the pathophysiological aspects of corticotroph tumor growth, with reference to the impact of adrenalectomy. The main epidemiological data on the Nelson's syndrome will be presented. More importantly, we will propose a new pathophysiological and practical approach to this question which attempts to evaluate the Corticotroph Tumor Progression after adrenalectomy, rather than to diagnose the Nelson's syndrome. We will discuss the consequences for the management of Cushing's disease patients after adrenalectomy, and will also draw some perspectives.

ACTH-Secreting Pituitary Adenoma↗
PubMed2005

Repeated remissions of Cushing's disease due to recurrent infarctions of an ACTH-producing pituitary macroadenoma.

Infarction of prolactin-secreting or growth hormone-secreting pituitary adenomas is not unusual. However, Infarction of ACTH-secreting adenomas has rarely been reported. Cyclical course of Cushing's syndrome alternating with adrenal insufficiency due to recurrent infarction of an ACTH-secreting pituitary adenoma has not been reported. We report here a 20-year-old lady who presented with florid signs of Cushing's syndrome but was found to have adrenal insufficiency on biochemical evaluation. Magnetic resonance imaging (MRI) of the pituitary gland showed that she had infarction of an ACTH-secreting macroadenoma. Over the next 6 years, her disease ran a cyclical course characterized by periods of hypercortisolism alternating with adrenal insufficiency due to repeated episodes of infarctions of the ACTH-secreting pituitary macroadenoma with corresponding changes in the pituitary adenoma on serial MRIs. The case alerts clinicians to this possibility when a patient presents with clinical picture of Cushing's syndrome but has adrenal insufficiency on biochemical testing. It also suggests that silent or subclinical infarction of pituitary adenomas is not uncommon and is probably under diagnosed.

Adenoma↗
PubMed2004

The association between calcitonin gene-related peptide (CGRP), substance P and headache in pituitary tumours.

OBJECTIVES: To determine if the differential expression of calcitonin gene-related peptide (CGRP) or substance P (SP) in a range of pituitary tumours was related to the presence or absence of headache. METHODS: Using recognised immunohistochemical techniques we examined twenty-six consecutive pituitary adenoma specimens for the presence of CGRP and SP. We included one normal post mortem pituitary specimen for comparison. A separate observer divided the patients into two groups: headache and non-headache. The association between the presence of CGRP, SP and headache was observed. RESULTS: We observed CGRP in seven specimens (27%) and SP in six tumour specimens (23%), with cytoplasmic staining being the predominant morphological picture. CGRP and SP were co-expressed in the same tumour specimen in five cases. There was no significant association between the presence of CGRP and headache (chi(2) 0.86; P = 0.35). We did not observe CGRP or SP in the control specimen. There was no correlation between tumour subtype and the presence of CGRP or SP. CONCLUSIONS: The mechanism of pituitary tumour-associated headache remains undetermined. The significance of the presence of CGRP and SP in pituitary tumours is unknown but does not appear to be related to headache or endocrine activity of the tumour.

Acromegaly↗
PubMed2005

Pituitary radiotherapy.

This article includes general comments about pituitary radiation, outcomes in patients who have Cushing's disease and adverse effects of treatment.

Humans↗
PubMed2005

Pituitary surgery and postoperative management in Cushing's disease.

Transsphenoidal pituitary surgery is the therapy for most Cushing's disease patients. This article describes the surgical technique, efficacy, perioperative management, and complications associated with this procedure. Numerous biochemical tests of cortisol status have been studied for the evaluation of the postoperative patient. Factors that predict postoperative remission and future relapse of Cushing's disease are addressed. Secondary interventions for persistent or recurrent disease include repeat transsphenoidal resection, pituitary radiation, medical therapy, and bilateral adrenalectomy

Humans↗
PubMed2005

Pediatric pituitary adenoma: a series of 42 patients.

Pituitary adenomas are uncommon in childhood. This report describes the presentation, endocrinological profile, management and outcome of 42 children with pituitary adenomas. The majority of the tumors were functioning adenomas (40/42, 95.2%). Only two patients (4.8%) had non-functioning tumors. Amongst the functioning tumors, there were 20 patients (47.6%) with prolactinomas, 11 patients (26.2%) with Cushing's disease and nine patients (21.4%) with growth hormone (GH)-secreting adenomas. The most common presenting features were endocrinopathy and visual disturbances. The majority of tumors were macroadenomas, with only eight patients (all with Cushing's disease) having a microadenoma. Transsphenoidal tumor decompression was performed in most cases (71.4%). Complications following transcranial surgery were more frequent when compared to transsphenoidal surgery. There was one death following surgery. Response to radiotherapy was satisfactory. Overall, 67.6% of patients achieved endocrinological remission. Of these, 89% of the children with GH-secreting tumors and 100% of the children with Cushing's disease achieved remission. We conclude that the transsphenoidal approach is effective and safe in surgery for pituitary adenomas in children and is the procedure of choice if there is no contraindication. The combination of surgery and radiotherapy, as well as medical therapy with bromocriptine, achieves good results in the management of these uncommon tumors.

Adenoma↗
PubMed2006

Myopathy in horses with pituitary pars intermedia dysfunction (Cushing's disease).

Fifteen horses with pituitary pars intermedia dysfunction were studied. The horses were of various breeds and between 15 and 28 years of age. Control horses matched for breed and age were studied for comparison. Evaluations included complete blood cell count and serum biochemical analysis, electromyography, and gluteus medius muscle biopsies for histochemical, morphometric, and ultrastructural analysis. No differences were found between groups of horses on routine laboratory analysis or electromyography. We demonstrated that muscle wasting in diseased horses was the result of atrophy of types 2A and 2B muscle fibers and loss of type 2B myofibers. Mild non-specific non-inflammatory myopathic alterations such as myofiber size variation, internal nuclei, perimysial, endomysial and sarcoplasmic fat accumulation were observed. At the ultrastructural level, subsarcolemmal mitochondrial accumulation and increased lipid droplets were evident. Similar to other species, this study confirmed atrophy of type 2 fibers as the cause of muscle mass loss in horses with Cushing's disease.

Animals↗