Paratesticular Tumours in a Patient with Nelson&amp;rsquo;s Syndrome

Ntalles, K.; Kostoglou-Athanassiou, Ifigenia; Georgiou, Eleana; Ikkos, D.

doi:10.1159/000184808

Cited by 12 publications

(8 citation statements)

References 12 publications

(31 reference statements)

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“…Paratesticular and paraovarian tumors producing cortisol or androgens in an ACTH-dependent manner have been described. 5,82,107 During embryogenesis, adrenal cortical cells may migrate along the line of gonadal descent or become sequestered in the hilum of the testes, giving rise to adrenal rest tissue. In NS, this adrenal rest tissue may become stimulated.…”

Section: Neurosurg Focus / Volume 23 / September 2007mentioning

confidence: 99%

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Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management

Banasiak¹,

Malek²

2007

Neurosurgical FOCUS

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ELSON syndrome is a rare clinical manifestation of an enlarging pituitary adenoma that can occur following bilateral adrenalectomy performed in the treatment of CD. It is characterized by skin and mucus membrane hyperpigmentation and excess ACTH secretion. Diagnostic criteria for NS have been quite variable. Some authors 77 have defined NS as high levels of ACTH associated with skin hyperpigmentation after bilateral adrenalectomy regardless of pituitary enlargement, 80 whereas others consider the presence of an expanding pituitary adenoma causing visual field disturbance as a requirement for diagnosis. 114 With modern day imaging, and for the purposes of this review, corticotroph tumor progression and high ACTH have been proposed as a basis for the diagnosis of NS. 4 Historical Perspective The first suggestion that the adrenal gland was essential for life came from Brown-Séquard, who showed in the 1850s that bilateral adrenalectomy in animals caused death within a few days. 66 Harvey Cushing 13 was the first to link adrenal hypersecretion of cortisol to the presence of a pituitary tumor. The concept of the hypothalamic-pituitary-adrenal axis evolved further with the demonstration of the existence of ACTH-releasing factor in 1955 by Saffran and Schally. 98 The first case report of the characteristic triad of NS was published by Don Nelson in 1958. 78 He described a 33-year-old woman who developed marked skin hyperpigmentation, high plasma ACTH levels, and imaging evidence of a pituitary tumor (enlarged sella on skull radiographs) 3 years after bilateral adrenalectomy for CD. In 1960, Nelson and colleagues 79 formally described the syndrome of pituitary hypersecretion of ACTH and pituitary tumor enlargement, which has since become the eponymous syndrome. Corticotropin-releasing hormone was finally isolated and sequenced by Vale and coworkers in 1981, 113 and with the availability of radioimmunoassay, the elevation of plasma ACTH levels after bilateral adrenalectomy became diagnostic for NS. Historically, patients with CD were treated with bilateral

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Section: Neurosurg Focus / Volume 23 / September 2007mentioning

confidence: 99%

“…Rarely, the adrenal rest tissue can produce sufficient cortisol to normalize levels or even cause recurrence of CD. 38,82,100 Predictive Risk Factors. Much effort has been directed towards the identification of predictive factors for the development of NS.…”

Section: Neurosurg Focus / Volume 23 / September 2007mentioning

confidence: 99%

Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management

Banasiak¹,

Malek²

2007

Neurosurgical FOCUS

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“…Paratesticular and paraovarian tumors producing cortisol or androgens in an ACTH-dependent manner have been described [53][54][55], as well as testicular adrenal rests with hyperplasia provoked bilateral testicular tumors [56,37].…”

Section: Complicationsmentioning

confidence: 99%

The Nelson's syndrome... revisited

et al. 2004

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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.

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“…The ambiguity of adrenal and gonad-specific gene expression is lost in adult life, but it may revert back in special situations such as during tumourigenesis. This may occur in gonads in the presence of high ACTH levels (Nelson's syndrome) (30)(31)(32)(33) and in the adrenal gland in the presence of high gonadotrophin levels (after gonadectomy or menopause) (16,17,34). Adrenal rest tumours in the gonad are assumed to arise from ACTH stimulation of adrenal tissue sequestered in the gonad during embryonic development, but it is also possible that they arise from undefined common adreno-gonadal stem cells in response to high ACTH stimulation.…”

Section: Discussionmentioning

confidence: 99%

The adrenal gland may be a target of LH action in postmenopausal women

et al. 2006

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Objective: LH receptor expression and function have been demonstrated in the human adrenal cortex, but their involvement in normal adrenal function remains elusive. Because cortisol levels have been reported to be higher in postmenopausal women than in age-matched men, the aim of the present study was to investigate a possible association of adrenal function with the elevated LH levels in postmenopausal women. Design and methods: A group of 112 endocrinologically normal postmenopausal women (mean age 67.6, range 50-88 years) was evaluated. A basal fasting morning sample of peripheral blood was taken for the determination of LH, cortisol, dehydroepiandrosterone-sulphate (DHEA-S), oestradiol (E2), testosterone, sex hormone-binding globulin (SHBG), insulin and glucose. Information about reproductive function, anthropometric parameters and arterial blood pressure was recorded. Results: The correlation of LH and cortisol was bimodally distributed, with a significant linear correlation up to the LH level of 41 U/l (nZ78, P!0.01), after which the increase of cortisol levelled off. Significant associations were also found between serum DHEA-S and LH levels (P!0.05), as well as between cortisol and testosterone (P!0.0001), but not between E2 and LH. Multivariate analysis showed that the association of cortisol with LH was independent of age and testosterone; the association of DHEA-S with LH was independent of E2, cortisol and age. Significant associations were also found between E2, testosterone and DHEA-S levels (P!0.001). Conclusions: These results indicate that adrenal cortisol and DHEA-S production may be stimulated by the highly elevated postmenopausal levels of LH; the physiological significance of this association and plausible contribution to the metabolic syndrome observed after the menopause remain to be evaluated. European Journal of Endocrinology 154 875-881

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Paratesticular Tumours in a Patient with Nelson’s Syndrome

Cited by 12 publications

References 12 publications

Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management

Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management

The Nelson's syndrome... revisited

The adrenal gland may be a target of LH action in postmenopausal women

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