Pediatric Cushing’s disease: Dichotomy in lateralization between imaging and inferior petrosal sinus sampling with review of literature

Abstract. Pre-operative bilateral simultaneous inferior petrosal sinus sampling with assessment of ACTH levels in the left and right sinuses and the periphery was performed in 9 patients with pituitary dependent Cushing's disease who were subsequently found at surgery to have basophil microadenomata. The novel observation of this study was the pattern of secretion of other pituitary hormones so that significant inter-sinus gradients ≥ 1.4:1 were seen for β-endorphin (2.8 ± 1.3, mean ± sem), PRL (4.2 ± 1.3) and GH (6.9 ± 2.4) as well as for ACTH (5.1 ± 1.1). There was no inter-sinus gradient for LH, FSH and TSH. In these 9 patients with adenomata, the correlations between the inter-sinus gradients for ACTH and β-endorphin were r = 0.95 (P <0.01), ACTH and PRL r = 0.90 (P < 0.01) and for ACTH and GH r = 0.89 (P <0.05). This close association between the gradients for ACTH and other anterior pituitary hormones could be due either to cosecretion of β-endorphin, PRL and GH by the ACTH-producing pituitary adenomata or to a paracrine effect of β-endorphin from the tumours on adjacent pituitary tissue. By reflecting the central pituitary hormone milieu, petrosal sinus sampling can give information about pituitary function unobtainable from peripheral hormone levels.

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

Neurosurgical FOCUS ◽

10.3171/2014.12.focus14780 ◽

2015 ◽

Vol 38 (2) ◽

pp. E7 ◽

Cited By ~ 9

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.

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Erratum. Value of pituitary gland MRI at 7 T in Cushing's disease and relationship to inferior petrosal sinus sampling: case report

Journal of Neurosurgery ◽

10.3171/2018.4.jns171969a ◽

2019 ◽

Vol 130 (2) ◽

pp. 661

Author(s):

Meng Law

Keyword(s):

Case Report ◽

Pituitary Gland ◽

Cushing’S Disease ◽

Cushing's Disease ◽

Inferior Petrosal Sinus ◽

Inferior Petrosal Sinus Sampling

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

Neurosurgical FOCUS ◽

10.3171/foc.2007.23.3.3 ◽

2007 ◽

Vol 23 (3) ◽

pp. 1-6 ◽

Cited By ~ 17

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.

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