McCune-Albright Syndrome and Acromegaly: Effects of Hypothalamopituitary Radiotherapy and/or Pegvisomant in Somatostatin Analog-Resistant Patients

Galland, F.; Kamenický, Peter; Affres, Hélène; Reznik, Yves; Pontvert, D.; Bouc, Yves Le; Young, Jacques; Chanson, Philippe

doi:10.1210/jc.2006-0561

Cited by 54 publications

(28 citation statements)

References 16 publications

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“…Treatment of GH-producing tumors in MAS with cabergoline has consistently shown an inadequate response, while long-acting octreotide has demonstrated an intermediate response. Recently, GH-receptor antagonists have been proposed as effective medical intervention for patients with inoperable MAS pituitary tumors or hypersomatotropinemia without a visible tumor [72,75,115].…”

Section: Molecular Genetics Of Pituitary Tumorsmentioning

confidence: 99%

Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics

Keil

Stratakis

2008

Expert Review of Neurotherapeutics

122

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Pituitary tumors are rare in childhood and adolescence, with a reported prevalence of up to 1 per million children. Only 2 -6% of surgically treated pituitary tumors occur in children. Although pituitary tumors in children are almost never malignant and hormonal secretion is rare, these tumors may result in significant morbidity. Tumors within the pituitary fossa are of two types mainly, craniopharyngiomas and adenomas; craniopharyngiomas cause symptoms by compressing normal pituitary, causing hormonal deficiencies and producing mass effects on surrounding tissues and the brain; adenomas produce a variety of hormonal conditions such as hyperprolactinemia, Cushing disease and acromegaly or gigantism. Little is known about the genetic causes of sporadic lesions, which comprise the majority of pituitary tumors, but in children, more frequently than in adults, pituitary tumors may be a manifestation of genetic conditions such as multiple endocrine neoplasia type 1 (MEN 1), Carney complex, familial isolated pituitary adenoma (FIPA), and McCune-Albright syndrome. The study of pituitary tumorigenesis in the context of these genetic syndromes has advanced our knowledge of the molecular basis of pituitary tumors and may lead to new therapeutic developments.

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Section: Molecular Genetics Of Pituitary Tumorsmentioning

confidence: 99%

Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics

Keil

Stratakis

2008

Expert Review of Neurotherapeutics

122

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“…Reported basal GH serum concentrations are the mean values for at least four serum samples obtained during an hourly profile. Serum IGF1 concentrations were measured as described by Mercado et al (24). Briefly, until 1998, sera were gel filtered on Ultrogel AcA 54 columns in acetic acid.…”

Section: Hormone Measurementsmentioning

confidence: 99%

D3 GH receptor polymorphism is not associated with IGF1 levels in untreated acromegaly

Kamenický

Santos

Espinosa

et al. 2009

European Journal of Endocrinology

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Context: A discrepancy between serum GH and IGF1 concentrations is frequent in patients with acromegaly. Here, we examined whether the exon 3-deleted (d3) GH receptor (GHR) variant, which has been linked to increased responsiveness to GH treatment in short children, influences the GH/IGF1 relationship in patients with acromegaly. Objective: To study the possible influence of the GHR genotype on the GH/IGF1 relationship in untreated acromegalic patients. Design: GHR genotype analysis with retrospective clinical and biochemical data collection performed in a single third-reference medical center. Patients and methods: Clinical data were obtained from the medical records of 105 acromegalic patients who had GH and IGF1 assays in the same laboratory and who were genotyped for the full-length (fl) or d3-GHR alleles. Results: The distribution of GHR genotypes was 51% fl/fl, 30% fl/d3, and 19% d3/d3. Patients with d3/d3 genotype were younger than the patients in the other two groups (P!0.05). Baseline GH and IGF1 concentrations did not differ among the three groups. The linear correlation between GH and IGF1 concentrations was similar in the three genotypic groups. Conclusions: The exon 3 GHR genotype does not affect the GH/IGF1 relationship in untreated acromegalic patients with high circulating GH and IGF1 levels.

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“…Although bromocrpitin was used in previous years and provided a reduction in both prolactin and GH levels, long-acting somatostatin analogues are preferred more frequently (3). However, better results have been obtained with combination of cabergolin which is a dopamin agonist and octreotide (11). Use of a single drug generally does not provide sufficient control.…”

Section: Discussionmentioning

confidence: 99%

McCune Albright syndrome in association with excessive GH secretion: case report

Özsu¹,

Mutlu²,

Çizmecioğlu³

et al. 2015

Turk Pediatri Ars

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McCune-Albright Syndrome is a rare syndrome characterized with excessive function of peripheral endocrine organs and activating mutations of the stimulatory G protein alpha subunit are involved in the pathogenesis. The three main findings of the disease include hyperpigmented café au lait spots, fibrous dysplasia and increased endocrine functions and excessive secretion of growth hormone is observed in 21% of the patients. Clinical signs may be missed in these patients because of precocious puberty and craniofacial fibrous dysplasia. Since radiotherapy causes to sarcomatous changes and transsphenoidal surgery may cause to severe thickening in the cranial bones, they are not appropriate treatment options and medical treatment is recommended. Bromocriptine, cabergoline and octreotide or different combinations of these drugs are used in treatment and pegvisomant has also been used in recent years. Here, we present a male patient aged 12 years and 7 months to show gigantism as a rare clinical reflection of McCune-Albright Syndrome with an excessive height (197 cm), café au lait spots, growht hormone levels which could not be supressed with oral glucose tolerance test and increased prolactin levels. (Turk Pediatri Ars 2015; 50: 114-7)

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McCune-Albright Syndrome and Acromegaly: Effects of Hypothalamopituitary Radiotherapy and/or Pegvisomant in Somatostatin Analog-Resistant Patients

Cited by 54 publications

References 16 publications

Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics

Pituitary tumors in childhood: update of diagnosis, treatment and molecular genetics

D3 GH receptor polymorphism is not associated with IGF1 levels in untreated acromegaly

McCune Albright syndrome in association with excessive GH secretion: case report

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