scholarly journals Cyclic Cushing's disease with misleading inferior petrosal sinus sampling results during a trough phase

2015 ◽  
Vol 38 (2) ◽  
pp. E7 ◽  
Author(s):  
Vivien Bonert ◽  
Namrata Bose ◽  
John D. Carmichael
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing’S Disease ◽  
Adrenocorticotropic Hormone ◽  
Diagnostic Testing ◽  
Cushing's Syndrome ◽  
Cushing's Disease ◽  
Inferior Petrosal Sinus ◽  
Inferior Petrosal Sinus Sampling ◽  
Acth Secreting ◽  
Transsphenoidal Resection

Diagnosing Cushing's syndrome is challenging and is further hampered when investigations are performed in a patient with cyclic Cushing's syndrome. A subset of patients with Cushing's syndrome exhibit periods of abnormal cortisol secretion with interspersed normal secretion. Patients can have periods of clinical improvement during these quiescent phases or remain symptomatic. Initial diagnostic testing can be challenging because of the unpredictable durations of the peak and trough phases, and it is especially challenging when the diagnosis of cyclic Cushing's syndrome has not yet been determined. Here, the authors present the case of a patient with Cushing's disease with a pathology-proven adrenocorticotropic hormone (ACTH)–secreting pituitary adenoma and whose initial inferior petrosal sinus sampling (IPSS) results were deemed indeterminate; further studies elucidated the diagnosis of cyclic Cushing's syndrome. Repeat IPSS was diagnostic of a central source for ACTH secretion, and the patient was treated successfully with transsphenoidal resection. Literature concerning the diagnosis and management of cyclic Cushing's syndrome is also reviewed.

scholarly journals The role of inferior petrosal sinus sampling in the diagnostic localization of Cushing's disease

2007 ◽  
Vol 23 (3) ◽  
pp. 1-6 ◽  
Author(s):  
Shivanand P. Lad ◽  
Chirag G. Patil ◽  
Edward R. Laws ◽  
Laurence Katznelson
Keyword(s):  
Pituitary Adenoma ◽  
Cushing’S Syndrome ◽  
Cushing’S Disease ◽  
Treatment Decisions ◽  
Cushing's Syndrome ◽  
Cushing's Disease ◽  
Inferior Petrosal Sinus ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Inferior Petrosal Sinus Sampling

✓ Cushing's syndrome can present a complex problem of differential diagnosis. Of cases in which hypercortisolemia results from an adrenocorticotropic hormone (ACTH)–dependent process, approximately 80% are due to a pituitary adenoma (Cushing's disease [CD]), 10% are due to adrenal lesions, and the remaining 10% are secondary to ectopic ACTH secretion. For patients with CD, surgical removal of the pituitary adenoma is the treatment of choice. Thus, localization of the source of ACTH secretion is critical in guiding timely treatment decisions. Inferior petrosal sinus sampling (IPSS) is considered to be the gold standard for confirming the origin of ACTH secretion in patients with Cushing's syndrome. The authors present an overview of IPSS—both the technique and its interpretation—as well as a summary of recent studies. A number of other techniques are discussed including sampling from the cavernous sinus, the jugular vein, and multiple sites to aid the diagnosis and lateralization of ACTH-producing pituitary adenomas. Management is best undertaken by a comprehensive multidisciplinary team taking into account the results of all the biochemical and imaging studies available, to provide the best advice in patient treatment decisions.

False-positive inferior petrosal sinus sampling in the diagnosis of Cushing's disease

1995 ◽  
Vol 83 (6) ◽  
pp. 1087-1091 ◽  
Author(s):  
Yoshihiro Yamamoto ◽  
Dudley H. Davis ◽  
Todd B. Nippoldt ◽  
William F. Young ◽  
John Huston ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
False Positive ◽  
Adrenocorticotropic Hormone ◽  
Pituitary Tumors ◽  
Cushing's Disease ◽  
Inferior Petrosal Sinus ◽  
Acth Secretion ◽  
Content Type ◽  
Inferior Petrosal Sinus Sampling ◽  
Acth Secreting

✓ Inferior petrosal sinus sampling (IPSS) for adrenocorticotropic hormone (ACTH) levels in patients with Cushing's syndrome has become a useful method to distinguish ACTH-secreting pituitary tumors (Cushing's disease) from other causes of the syndrome, principally ectopic adrenocorticotropin secretion by an occult tumor. Although the test is generally regarded as highly specific, the authors recently encountered two patients whose IPSS measurements were false-positive for Cushing's disease. The results of IPSS suggested a pituitary origin of ACTH secretion in both patients, but transsphenoidal surgery failed to disclose a pituitary adenoma or to improve postoperative plasma cortisol levels. Both patients subsequently were found to have an ACTH-secreting carcinoid tumor of the lung. The false-positive IPSS studies were due to periodic hormonogenesis. The patients must be hypercortisolemic at the time IPSS is performed for the study to be valid.

scholarly journals The role of bilateral inferior petrosal sinus sampling in the diagnosis of Cushing's syndrome

2007 ◽  
Vol 51 (8) ◽  
pp. 1329-1338 ◽  
Author(s):  
Andrea Utz ◽  
Beverly M.K. Biller
Keyword(s):  
Cushing’S Syndrome ◽  
Venous Drainage ◽  
Cushing's Syndrome ◽  
Blood Levels ◽  
Inferior Petrosal Sinus ◽  
Ectopic Acth ◽  
Inferior Petrosal Sinus Sampling ◽  
Neoplastic Lesion ◽  
Before And After ◽  
Acth Secreting

Adrenocorticotropin hormone (ACTH)-dependent Cushing's syndrome is most often due to a pituitary corticotroph adenoma, with ectopic ACTH-secreting tumors representing approximately 15% of cases. Biochemical and radiological techniques have been established to help distinguish between these two entities, and thus aid in the localization of the neoplastic lesion for surgical resection. The test that offers the highest sensitivity and specificity is bilateral inferior petrosal sinus sampling (BIPSS). BIPSS is an interventional radiology procedure in which ACTH levels obtained from venous drainage very near the pituitary gland are compared to peripheral blood levels before and after corticotropin hormone (CRH) stimulation. A gradient between these two locations indicates pituitary Cushing's, whereas the absence of a gradient suggests ectopic Cushing's. Accurate BIPSS results require hypercortisolemia to suppress normal corticotroph ACTH production and hypercortisolemia at the time of the BIPSS to assure excessive ACTH secretion. In some cases, intrapituitary gradients from side-to-side can be helpful to localize small corticotroph adenomas within the sella. BIPSS has rare complications and is considered safe when performed at centers with experience in this specialized technique.

scholarly journals Dexmedetomidine for Conscious Sedation in Bilateral Inferior Petrosal Sinus Sampling

2016 ◽  
Vol 1 (2) ◽  
pp. 61-62
Author(s):  
Neeraj Barnwal ◽  
Raylene Dias ◽  
Rahul Mamde
Keyword(s):  
General Anesthesia ◽  
Cushing’S Syndrome ◽  
Conscious Sedation ◽  
Cushing's Syndrome ◽  
Inferior Petrosal Sinus ◽  
The Novel ◽  
Rare Presentation ◽  
Inferior Petrosal Sinus Sampling ◽  
Ectopic Adrenocorticotropic Hormone ◽  
Acth Secreting

ABSTRACT Cushing's syndrome in an acromegalic patient is a very rare presentation. To differentiate a Cushing's disease from Cushing's syndrome due to ectopic adrenocorticotropic hormone (ACTH) secreting tumors, inferior petrosal sinus sampling (IPSS) is required. Acromegaly patients have associated airway abnormalities posing a challenge to administration of anesthesia. Traditionally, most IPSS was done under general anesthesia. But now it is being recognized that general anesthesia for this procedure has its own implications and hence conscious sedation is being used for this purpose. We describe our experience with the novel agent dexmedetomidine for conscious sedation in this procedure. How to cite this article Barnwal N, Dias R, Mamde R. Dexmedetomidine for Conscious Sedation in Bilateral Inferior Petrosal Sinus Sampling. Res Inno in Anesth 2016;1(2):61-62.

scholarly journals The role of inferior petrosal sinus sampling in ACTH-dependent Cushing's syndrome: review and joint opinion statement by members of the Italian Society for Endocrinology, Italian Society for Neurosurgery, and Italian Society for Neuroradiology

2015 ◽  
Vol 38 (2) ◽  
pp. E5 ◽  
Author(s):  
Francesca Pecori Giraldi ◽  
Luigi Maria Cavallo ◽  
Fabio Tortora ◽  
Rosario Pivonello ◽  
Annamaria Colao ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Adrenocorticotropic Hormone ◽  
Dynamic Testing ◽  
Cushing's Syndrome ◽  
Italian Society ◽  
The Other ◽  
Inferior Petrosal Sinus ◽  
Etiological Diagnosis ◽  
Inferior Petrosal Sinus Sampling

In the management of adrenocorticotropic hormone (ACTH)–dependent Cushing's syndrome, inferior petrosal sinus sampling (IPSS) provides information for the endocrinologist, the neurosurgeon, and the neuroradiologist. To the endocrinologist who performs the etiological diagnosis, results of IPSS confirm or exclude the diagnosis of Cushing's disease with 80%–100% sensitivity and over 95% specificity. Baseline central-peripheral gradients have suboptimal accuracy, and stimulation with corticotropin-releasing hormone (CRH), possibly desmopressin, has to be performed. The rationale for the use of IPSS in this context depends on other diagnostic means, taking availability of CRH and reliability of dynamic testing and pituitary imaging into account. As regards the other specialists, the neuroradiologist may collate results of IPSS with findings at imaging, while IPSS may prove useful to the neurosurgeon to chart a surgical course. The present review illustrates the current standpoint of these 3 specialists on the role of IPSS.

Hyperfunctioning unilateral adrenal macronodule in three patients with Cushing's disease: hormonal and imaging characterization

1993 ◽  
Vol 129 (4) ◽  
pp. 284-290 ◽  
Author(s):  
Roger Abs ◽  
Frank Nobels ◽  
Johan Verhelst ◽  
Philippe Chanson ◽  
Charles Mahler ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Ct Scanning ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
Cushing's Disease ◽  
Cortisol Secretion ◽  
Inferior Petrosal Sinus ◽  
Plasma Acth ◽  
Inferior Petrosal Sinus Sampling ◽  
Adrenal Nodule

We aimed to investigate the dynamics of adrenocorticotropin (ACTH) and cortisol secretion in pituitary-dependent Cushing's syndrome with bilateral macronodular adrenal hyperplasia presenting as a single adrenal macronodule, and to determine the imaging characteristics of this syndrome. Three female patients were studied. Plasma ACTH and serum cortisol secretion were studied by determining their rhythmicity and pulsatility and their responses to the administration of ovine corticotropin-releasing factor, thyrotropin-releasing hormone, metyrapone, tetracosactrin, insulin and dexamethasone. Techniques used to localize the anatomical lesion were bilateral simultaneous inferior petrosal sinus sampling, magnetic resonance examination of the pituitary, computed tomography (CT) scanning and [75Se]cholesterol scintigraphy of the adrenal glands. Plasma ACTH and serum cortisol levels were measured using a commercial radioimmunoassay and an immunoradiometric assay. The ACTH and cortisol pulse number and amplitude were calculated using established computer software. In all three patients ACTH and cortisol secretory dynamics fulfilled the requirements for diagnosis of pituitary-dependent Cushing's syndrome. A close relationship between ACTH and cortisol pulses also favored a pituitary dependency. Study of the amplitude of cortisol pulses classified two patients in the group of hypopulsatile Cushing's disease. Adrenal CT scanning demonstrated the presence of a large single nodule. [75Se]Cholesterol scintigraphy showed bilateral radionuclide uptake, although mostly localized over the adrenal nodule. All patients underwent successful trans-sphenoidal hypophysectomy. Over a period of 1 year, a slow shrinkage of the adrenal nodule was observed in two patients, while no change in volume was observed in one patient. Demonstration of an adrenal macronodule on CT scanning in patients with Cushing's syndrome is in itself insufficient to allow the diagnosis of hypercorticism due to a unilateral adrenal adenoma. Additional dynamic endocrine testing, inferior petrosal sinus sampling and imaging techniques such as [75Se]cholesterol scintigraphy remain necessary to reach a correct diagnosis.

scholarly journals MON-178 Cyclical Cushing’s Syndrome in 12 Patients with Ectopic ACTH Secretion

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Anna Zenno ◽  
Raven McGlotten ◽  
Atil Kargi ◽  
Lynnette Nieman
Keyword(s):  
Medical Treatment ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Inferior Petrosal Sinus ◽  
Acth Secretion ◽  
Diagnostic Challenge ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Inferior Petrosal Sinus Sampling ◽  
Acth Secreting

Abstract Background: Cyclical Cushing’s syndrome (CCS) is characterized by alternating periods of endogenous hypercortisolism and eucortisolism. A literature survey of 60 adult patients with CCS found 15 to have ectopic ACTH secretion (EAS) (1). The duration and frequency of hypercortisolemia are unpredictable, creating a diagnostic challenge. Objective: Describe biochemical and clinical characteristics of patients with CCS due to occult or histologically proven ectopic ACTH-secreting neuroendocrine tumor (NET). Methods: We conducted a retrospective medical record review of 12 adults with EAS admitted to our institution. Inclusion required 1) evidence of ectopic ACTH tumor from biochemical testing (CRH stimulation, 8 mg dexamethasone suppression [DST], and/or inferior petrosal sinus sampling [IPSS]) or pathology results and 2) cycles of hypercortisolism (Hi-F) to eucortisolism (Eu-F) off medical treatment. Results: Average age on admission was 61 (46-79) years; 58% were women. All 12 had biochemical evidence of ACTH-dependent Hi-F. IPSS results suggested EAS in 9 patients, 8 of whom had Hi-F for more than two months, and 1 whose cycles occurred every 5 - 7 days. IPSS was consistent with Cushing’s disease (CD) in 2 patients after Hi-F of only 6 -7 weeks and one with Eu-F on admission, estimated duration < 4 weeks. DST suggested EAS in 9 patients, and CD in the one with recent Eu-F. CRH was consistent with EAS in 10 patients, but suggested CD in 2 with marginal increases in ACTH (34.5%, 38%) but not cortisol. 7 patients had ACTH-secreting tumor on pathology (5 pulmonary, 1 pancreas, 1 appendix NET), and 5 had occult presumed EAS. Time from one Hi-F episode to the next ranged from 1 week to 6 years with Hi-F duration of 3 days to 5 years. 24-hour urine free cortisol (UFC) levels were 17 - 301 times the upper reference range (RR) during Hi-F periods. During Eu-F, lowest UFCs were within RR in 9 patients and subnormal in 3. Hypokalemia occurred in 11 patients with Hi-F; increasing values paralleled movement to Eu-F. Conclusion: Patients with possible ectopic ACTH-secretion and CCS may pose a diagnostic challenge: clinical and biochemical evidence of hypercortisolemia may not be present, depending on the timing and/or duration of hypercortisolism. Furthermore, test results may inappropriately suggest Cushing’s disease if performed after less than 8 weeks of hypercortisolism, or with recent eucortisolism. Thus, weekly UFC measurement may facilitate diagnosis of cyclical Cushing’s syndrome and determine appropriate timing of dynamic testing such as inferior petrosal sinus sampling. Potassium may be a useful marker to determine when medical treatment can be tapered or stopped. 1. Meinardi JR, et al. Eur J Endocrinol. 157:245, 2007.

scholarly journals Systemic Sarcoidosis Unmasked by Cushing’s Disease Surgical Treatment

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Daniele Bongetta ◽  
Cesare Zoia ◽  
Francesco Lombardi ◽  
Elisabetta Lovati ◽  
Pietro Lucotti ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Pituitary Adenoma ◽  
Cushing’S Syndrome ◽  
Cushing’S Disease ◽  
Cushing's Syndrome ◽  
Cushing's Disease ◽  
Systemic Sarcoidosis ◽  
Acth Secreting ◽  
Possible Cause

Diseases responsive to glucocorticoids, like sarcoidosis, are rarely masked by Cushing’s syndrome. An ACTH secreting pituitary adenoma is a possible cause of Cushing’s syndrome and its resection can make a subclinical sarcoidosis clear. Only few cases of sarcoidosis following the treatment of hypercortisolism are reported in literature. We report a case of sarcoidosis after the resection of an ACTH secreting pituitary adenoma.

Cushing's Disease in a Young Woman with Anorexia Nervosa: Pathophysiological Implications

1986 ◽  
Vol 31 (9) ◽  
pp. 861-864 ◽  
Author(s):  
Jack L. Katz ◽  
Herbert Weiner ◽  
Jacob Kream ◽  
Barnett Zumoff
Keyword(s):  
Anorexia Nervosa ◽  
Pituitary Adenoma ◽  
Young Woman ◽  
Cushing’S Syndrome ◽  
Cushing’S Disease ◽  
Adrenal Glands ◽  
Cushing's Syndrome ◽  
Cushing's Disease ◽  
Sequence Of Events ◽  
Acth Secreting

This report describes a 17-year old student who was found to have Cushing's syndrome two years after she had developed anorexia nervosa (AN). The Cushing's syndrome was treated with bilateral resection of enlarged, hyperplastic, non-tumoraus adrenal glands. The diagnosis was further confirmed four years later when, two to three years after new symptoms had appeared, an ACTH secreting pituitary adenoma (that is, Cushing's disease) was found on surgery. The possible mechanism for the development of Cushing's disease in a patient with prior anorexia nervosa, a sequence of events reported once previously, is discussed. It is suggested that increased hypothalamić-pituitary corticotroph stimulation in association with the anorexia nervosa, a now well-established endocrine phenomenon, activated an occult, inactive pituitary basophil adenoma in this patient, eventually resulting in autonomous pituitary overproduction of ACTH by the tumor.

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