Paediatric Pituitary Adenomas: A Decade of Change

Guaraldi, Federica; Storr, Helen L; Ghizzoni, Lucia; Ghigo, Ezio; Savage, Martin O.

doi:10.1159/000357673

Cited by 58 publications

(52 citation statements)

References 58 publications

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“…The diagnosis of an ACTH-secreting pituitary adenoma follows from the investigation of suspected CS and the demonstration of ACTH-dependent hypercortisolaemia. Our published protocol for diagnosis of CD, in which the initial screening tests have a high sensitivity, is given in Table 2 (7,25). have a reported sensitivity of 88% and specificity of 90% (26), suggesting that UFC alone may not be an ideal screening test.…”

Section: Biochemical Evaluationmentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Paediatric Cushing's disease

Storr

Savage

2015

European Journal of Endocrinology

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Cushing's disease (CD) is the commonest form of ACTH-dependent Cushing's syndrome and is a rare clinical diagnosis in paediatric and adolescent patients. CD is caused by an ACTH-secreting pituitary corticotroph adenoma and is associated with significant morbidity in children; therefore, early diagnosis and treatment are critical for optimal therapeutic outcome. This review highlights the key clinical and biochemical features of paediatric CD and appraises current practices in diagnosis and management. A close liaison with adult endocrinology colleagues, particularly, for interpretation of investigations and definition of therapeutic strategy is strongly advised.

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Section: Biochemical Evaluationmentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Paediatric Cushing's disease

Storr

Savage

2015

European Journal of Endocrinology

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“…The differential diagnosis of central precocious puberty includes arachnoid cysts, ependymomas, germ cell tumors, low-grade gliomas, craniopharyngiomas, hypothalamic hamartomas, hydrocephalus, radiation to the CNS, primary hypothyroidism and genetic causes such as gain-of-function mutations of the kisspeptin gene as well as loss-of-function mutations of the MKRN3 gene (5). Pituitary adenomas in general and FGA in particular are very rare in childhood (6). Although still very infrequent, isosexual central precocious puberty due to FGA is somewhat more frequent in males than in females (3).…”

Section: Discussionmentioning

confidence: 99%

An FSH and TSH pituitary adenoma, presenting with precocious puberty and central hyperthyroidism

Vargas

Balcázar-Hernández

Melgar

et al. 2017

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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A 19-year-old woman with a history of isosexual precocious puberty and bilateral oophorectomy at age 10 years because of giant ovarian cysts, presents with headaches and mild symptoms and signs of hyperthyroidism. Hormonal evaluation revealed elevated FSH and LH levels in the postmenopausal range and free hyperthyroxinemia with an inappropriately normal TSH. Pituitary MRI showed a 2-cm macroadenoma with suprasellar extension. She underwent successful surgical resection of the pituitary tumor, which proved to be composed of two distinct populations of cells, each of them strongly immunoreactive for FSH and TSH, respectively. This mixed adenoma resulted in two different hormonal hypersecretion syndromes: the first one during childhood and consisting of central precocious puberty and ovarian hyperstimulation due to the excessive secretion of biologically active FSH and which was not investigated in detail and 10 years later, central hyperthyroidism due to inappropriate secretion of biologically active TSH. Although infrequent, two cases of isosexual central precocious puberty in girls due to biologically active FSH secreted by a pituitary adenoma have been previously reported in the literature. However, this is the first reported case of a mixed adenoma capable of secreting both, biologically active FSH and TSH.Learning points:Although functioning gonadotrophinomas are infrequent, they should be included in the differential diagnosis of isosexual central precocious puberty.Some functioning gonadotrophinomas are mixed adenomas, secreting other biologically active hormones besides FSH, such as TSH.Early recognition and appropriate treatment of these tumors by transsphenoidal surgery is crucial in order to avoid unnecessary therapeutic interventions that may irreversibly compromise gonadal function.

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“…Cushing’s disease is usually sporadic, yet may be familial, and is known to occur in the context of MEN-1 and rarely because of mutations of the AIP gene [20,21,22 ▪▪ ]. MEN-1 includes ACTH secreting pituitary adenomas in 5–10% of cases [23].…”

Section: Geneticsmentioning

confidence: 99%

Cushingʼs syndrome in childhood

Lodish¹

2015

Current Opinion in Endocrinology & Diabetes and Obesity

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Purpose of review To provide an update on the genes associated with Cushing’s syndrome in children, as well as to familiarize the clinician with recent treatment guidelines and outcome data for children with Cushing’s syndrome. Recent findings The list of genes associated with Cushing’s syndrome continues to grow. In addition, treatment for childhood Cushing’s syndrome is evolving. As long-term follow-up data on children becomes available, clinicians need to be aware of the issues that require attention. Summary Knowledge of the specific genetic causes of Cushing’s syndrome has potential implications for treatment, surveillance, and counseling. Advances in surgical technique, radiation modalities, and medical therapies offer the potential for additional treatment options in Cushing’s syndrome. Early identification and management of post-treatment morbidities in children treated for Cushing’s syndrome is crucial in order to optimize care.

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Paediatric Pituitary Adenomas: A Decade of Change

Cited by 58 publications

References 58 publications

MANAGEMENT OF ENDOCRINE DISEASE: Paediatric Cushing's disease

MANAGEMENT OF ENDOCRINE DISEASE: Paediatric Cushing's disease

An FSH and TSH pituitary adenoma, presenting with precocious puberty and central hyperthyroidism

Cushingʼs syndrome in childhood

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