McCune-Albright syndrome and acromegaly: hormonal control with use of cabergoline and long-acting somatostatin--case report.

OBJECTIVES: The use of drug therapy based on cabergoline, octreotide and long-acting release (LAR) octreotide has presented varying results in the treatment of GH excessive production in patients with McCune-Albright Syndrome. METHODS: We report the case of a 29 year-old female patient presenting McCune-Albright Syndrome and complaint of excessive bone growth. RESULTS: The patient presented a pituitary adenoma involving the right internal carotid artery and excessive secretion of growth hormone (no GH suppression was observed after the oral glucose tolerance test). Due to the presence of diffuse thickness in skull base bones, surgical approach was not considered effective and the patient was submitted to drug therapy with octreotide LAR and cabergoline. At the one year follow-up, GH and IGF-1 levels were normal and no adverse effects were present. CONCLUSION: The use of drug therapy based on the association of cabergoline and octreotide is safe and able to achieve complete hormonal control in the treatment of acromegaly for McCune-Albright patients.

McCune-albright syndrome and acromegaly: hormonal control with use of cabergoline and long-acting somatostatin - case report / Síndrome de McCune-albright associada à acromegalia: controle hormonal com cabergolina e somatostatina de longa duração - relato de caso

OBJECTIVES: The use of drug therapy based on cabergoline, octreotide and long-acting release (LAR) octreotide has presented varying results in the treatment of GH excessive production in patients with McCune-Albright Syndrome. METHODS: We report the case of a 29 year-old female patient presenting McCune-Albright Syndrome and complaint of excessive bone growth. RESULTS: The patient presented a pituitary adenoma involving the right internal carotid artery and excessive secretion of growth hormone (no GH suppression was observed after the oral glucose tolerance test). Due to the presence of diffuse thickness in skull base bones, surgical approach was not considered effective and the patient was submitted to drug therapy with octreotide LAR and cabergoline. At the one year follow-up, GH and IGF-1 levels were normal and no adverse effects were present. CONCLUSION: The use of drug therapy based on the association of cabergoline and octreotide is safe and able to achieve complete hormonal control in the treatment of acromegaly for McCune-Albright patients.

OBJETIVO: O uso de terapia medicamentosa, como cabergolina, octreotide e octreotide de longa duração, tem apresentado resultados variados no tratamento da produção excessiva de hormônio de crescimento (GH) em pacientes com síndrome de McCune-Albright. MÉTODOS: Foi relatado o caso de uma paciente de 29 anos apresentando síndrome de McCune-Albright com queixas de crescimento ósseo excessivo. RESULTADOS: A paciente apresentava adenoma pituitário com envolvimento da artéria carótida interna direita e produção excessiva de GH (sem supressão de GH após o teste de supressão com glicose). Por causa do aumento importante da espessura dos ossos da base do crânio, a abordagem cirúrgica foi considerada pouco efetiva e a paciente foi submetida à terapia medicamentosa com octreotide de longa duração e cabergolina. No seguimento de um ano, os níveis de GH e IGF-1 estavam normais e os efeitos adversos não eram presentes. CONCLUSÃO: A terapia medicamentosa fundamentada na associação de cabergolina e octreotide é segura e capaz de alcançar controle hormonal completo no tratamento de acromegalia na síndrome de McCune-Albright.

Outcome of surgical intrasellar growth hormone tumor performed by a pituitary specialist surgeon in a developing country.

BACKGROUND: Acromegaly is an excessive GH secretion, which in most cases, is caused by a pituitary GH-secreting adenoma. Traditional treatment of acromegaly consists of surgery, drug therapy, and eventually radiotherapy. The aim of this retrospective study is to evaluate the results of transsphenoidal endoscopic surgery in a group of patients with intrasellar GH adenoma who were operated by a pituitary specialist surgeon. We shall then argue about the economical advantages, for the NHS of a developing country, between surgical and medical treatment. METHODS: We have analyzed data from 33 patients with intrasellar GH tumor who had been referred to the neuroendocrine department of the HGF, Brazil. The patients underwent a transsphenoidal endoscopic adenomectomy for acromegaly between 2000 and 2005. Their ages were between 20 and 67 years (mean, 44 years) at the moment of surgery. No cavernous sinus invasion was present. Follow-up was a median of 2 years (range, 12 months-6 years). RESULTS: All 33 patients had intrasellar adenoma, 84.84% of patients achieved remission by surgery. One patient was operated twice and reached hormonal normalization. Five patients still had the disease and refused a second surgery. A treatment with octreotide was started for these 5 patients and resulted in an adequate control of GH and IGF-1 levels. No patients had radiotherapy. CONCLUSION: Our patients, with intrasellar GH tumor, operated by a pituitary specialist neurosurgeon had remission rates approaching those obtained by most specialized neurosurgical centers worldwide. For equal results, our study shows that the surgical treatment is the best issue for the patient and for the NHS.