Guidelines for Acromegaly Management

Melmed, Shlomo; Casanueva, Felipe F.; Cavagnini, Francesco; Chanson, Philippe; Frohman, Lawrence A.; Grossman, Ashley; Ho, Ken K. Y.; Kleinberg, David L.; Lamberts, S. W. J.; Laws, Edward R.; Lombardi, Gaetano; Vance, Mary Lee; Werder, Klaus von; Wass, John; Giustina, Andrea

doi:10.1210/jc.2002-011841

Cited by 322 publications

(196 citation statements)

References 53 publications

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“…Most CNC patients will have some abnormality of GH secretion due to the underlying pituitary hyperplasia, but almost all of them will have negative imaging studies [100,108]. It is common practice to treat CNC patients with elevated IGF-1 levels with somatostatin analogues with the goal of normalizing IGF-1 [100,109]. For CNC patients with abnormal response to oral glucose tolerance tests but normal IGF-1 levels and normal pituitary imaging, evaluations should be performed annually to assess for changes that may require treatment.…”

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

126

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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

126

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“…Octreotide is the most widely used medical treatment for acromegaly; it is effective in more than 50% of patients, is well tolerated and has high compliance rates based on monthly i.m. injections (1,7,8,15). However, in patients not achieving optimal IGF1 control on conventional SSA therapy, current guidelines recommend other treatment modalities (pegvisomant and/or radiosurgery in the case of post-surgical treatment or surgery for those receiving primary medical treatment) (1).…”

Section: Discussionmentioning

confidence: 99%

“…injections (1,7,8,15). However, in patients not achieving optimal IGF1 control on conventional SSA therapy, current guidelines recommend other treatment modalities (pegvisomant and/or radiosurgery in the case of post-surgical treatment or surgery for those receiving primary medical treatment) (1). Although alternative treatment modalities show higher efficacy rates than the high-dose octreotide regimen used in this study, they are generally associated with increased cost as well as potential side effects and inconvenience to the patient (9,(17)(18)(19).…”

Section: Discussionmentioning

confidence: 99%

“…Approximately, 25-50% of patients with this disease have an inadequate response to long-acting somatostatin analogues (SSAs). Current guidelines suggest switching those patients to other therapeutic modalities that are associated with higher costs and potential risks (1,2). The pathophysiological basis of poor SSA response is unclear, but thought to be associated with tumour cell SS receptor expression and SSA-related factors such as dose and dosing intervals (3).…”

Section: Introductionmentioning

confidence: 99%

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High-dose intramuscular octreotide in patients with acromegaly inadequately controlled on conventional somatostatin analogue therapy: a randomised controlled trial

Giustina¹,

Bonadonna²,

Bugari³

et al. 2009

European Journal of Endocrinology

113

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Objective: In acromegaly, 25-50% of patients respond inadequately to conventional long-acting somatostatin analogue (SSA) therapy. Response may be improved by increasing SSA frequency or dose. This study evaluated the biochemical efficacy and safety of high-dose octreotide in patients with acromegaly. Design: A 24-week prospective, multicentre, randomised, open-label trial conducted from 12 December 2005 to 23 October 2007 in patients with persistently uncontrolled acromegaly despite R6 month conventional SSA therapy. Methods: Patients with R50% reduction in GH levels during previous SSA treatment were randomised to high-dose (60 mg/28 days) or high-frequency (30 mg/21 days) octreotide i.m. injection. Primary end-points were week 12 and 24 reduction in serum IGF1 and GH from baseline. Secondary end points included IGF1 normalisation and tumour shrinkage rates, and safety/tolerability evaluations. Results: Significantly, more patients (10 out of 11) achieved week 24 IGF1 reduction in the high-dose than the high-frequency group (8 out of 15; P!0.05). In the high-dose group only, week-24 IGF1 values were significantly reduced (PZ0.02) versus baseline. Normalisation of IGF1 occurred only with the high-dose regimen (4/11; PZ0.02). Out of 14 patients experiencing adverse events, 5 reported drug-related gastrointestinal effects. No dose-response relationship was seen. Safety parameters were similar between treatment groups, apart from a slight decrease in HbA1c in the high-dose group only. Conclusion: High-dose octreotide treatment is safe and effective (normalisation of IGF1 levels) in a subset of patients with active acromegaly inadequately controlled with long-term SSA. Individualised octreotide doses up to 60 mg/28 days may improve outcomes of SSA therapy.

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“…T rans-sphenoidal surgery is the most common initial treatment approach of patients with somatotropinomas (1). However, somatotropinomas are more often expansive macroadenomas and surgery is usually not curative.…”

Section: Introductionmentioning

confidence: 99%

Somatostatin receptors subtypes 2 and 5, dopamine receptor type 2 expression and gsp status as predictors of octreotide LAR® responsiveness in acromegaly

Neto

Taboada

Gadelha

2008

Arq Bras Endocrinol Metab

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We present two acromegalic patients in which clinical and molecular data are discussed in regard to their ability to predict long term octreotide LAR ® therapy response. Case reports: Patient 1: female, 36 years old at diagnosis. Basal GH and IGF-I at diagnosis were 133 ng/mL and 181% above the upper limit of reference values (ULRV), respectively. Growth hormone during acute test with subcutaneous octreotide decreased from 133 to 13 ng/mL. Patient started on primary octreotide LAR ® therapy (20mg q28 days) and achieved biochemical parameters of disease control after 6 months. Molecular analysis of tumor fragments: gsp +; quantitative analysis of SSTR (somatostatin receptor) and DR (dopamine receptor) mRNA -SSTR2 23954; SSTR5 2407; DR2 total 17016 copies. Patient 2: male, 38 years old at diagnosis. Basal GH and IGF-I at diagnosis were 120 ng/mL and 114% ULRV, respectively. Patient underwent noncurative trans-sphenoidal surgery. Post-operative GH and IGF-I were 112 ng/ mL and 137% ULRV, respectively. Growth hormone during acute test with subcutaneous octreotide decreased from 112 to 7 ng/mL. Octreotide LAR ® therapy (20 mg q28 days) was then initiated. After 6 months of treatment, patient did not attain biochemical control of disease and displayed increased tumor volume. Molecular analysis of tumor fragments: gsp not done; quantitative analysis of SSTR and DR mRNA -SSTR2 416; SSTR5 3767; DR2 total 3439 copies. In conclusion, these two cases illustrate how laboratory data can be confl icting as predictors of octreotide LAR ® responsiveness and how molecular analysis of tumor fragments can help explain different behaviors in clinically similar patients. RESUMO Expressão dos Receptores da Somatostatina Subtipos 2 e 5 e do Receptor da Dopamina tipo 2 e gsp Status como Preditores de Resposta ao Octreotide LAR® na Acromegalia.Apresentamos dois pacientes acromegálicos nos quais dados clínicos e moleculares são discutidos quanto à sua capacidade de predizer a resposta a longo prazo ao tratamento com octreotide LAR ® . Relato dos casos: Paciente 1: Feminina, 36 anos de idade ao diagnóstico. GH e IGF-I ao diagnóstico 133 ng/mL e 181% acima do limite superior do valor de referência (LSVR), respectivamente. GH durante o teste agudo com octreotide subcutâneo diminuiu de 133 para 13 ng/mL. Foi iniciado tratamento primário com octreotide LAR ® (20 mg q28 dias) e a paciente alcançou os parâmetros bioquímicos de controle de doença depois de seis meses. Análise molecular do tumor: gsp +; análise quantitativa do mRNA de SSTR (receptores de somatostatina) e DR (receptor de dopamina) -SSTR2 23.954; SSTR5 2.407; DR2 total 17.016 có-pias. Paciente 2: Masculino, 38 anos de idade ao diagnóstico. GH e IGF-I ao diagnóstico 120 ng/mL e 114% LSVR, respectivamente. Paciente foi sub-

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Guidelines for Acromegaly Management

Cited by 322 publications

References 53 publications

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

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

High-dose intramuscular octreotide in patients with acromegaly inadequately controlled on conventional somatostatin analogue therapy: a randomised controlled trial

Somatostatin receptors subtypes 2 and 5, dopamine receptor type 2 expression and gsp status as predictors of octreotide LAR® responsiveness in acromegaly

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