RESUMO
CONTEXT: Acromegaly is a systemic disorder caused by a GH-secreting pituitary adenoma. As with other rare diseases, acromegaly registries developed in various European countries have provided us with important information. OBJECTIVE: The objective of the study was to analyze the epidemiological, clinical, biochemical, and therapeutic data from the Mexican Acromegaly Registry (MAR). SETTING: The setting of the study was a nationwide patient registry. DESIGN AND METHODOLOGY: The MAR was created in 2009. It gathers data from 24 participating centers belonging to three different institutions using a specifically designed on-line platform. Only patients diagnosed after 1990 were included in the program. RESULTS: A total of 2057 patients (51% female, mean age at diagnosis 41.1 ± 24.5 y) have been registered for an estimated prevalence of 18 cases per 1 million inhabitants. Hypertension, glucose intolerance, diabetes, and dyslipidemia were present in 27%, 18.4%, 30%, and 24% of the patients, respectively. The IGF-1 level at diagnosis and the concomitant presence of hypertension were significantly associated with the development of diabetes. Transsphenoidal surgery was the primary treatment in 72% of the patients. Pharmacological treatment, mostly with somatostatin analogs, was administered primarily and adjunctively in 26% and 54% of the patients, respectively. Treatment choice varied among the three participating institutions, with the predominance of pharmacological therapy in two of them and of radiation therapy in the third. Therapeutic outcomes were similar to those reported in the European registries. CONCLUSIONS: The MAR is the largest and first non-European registry of the disease. Our findings highlight important within-country differences in treatment choice due to variations in the availability of resources.
Assuntos
Acromegalia/etiologia , Adenoma/diagnóstico , Adenoma Hipofisário Secretor de Hormônio do Crescimento/diagnóstico , Hipófise/fisiopatologia , Acromegalia/prevenção & controle , Adenoma/epidemiologia , Adenoma/fisiopatologia , Adenoma/terapia , Adulto , Estudos de Coortes , Terapia Combinada , Feminino , Seguimentos , Adenoma Hipofisário Secretor de Hormônio do Crescimento/epidemiologia , Adenoma Hipofisário Secretor de Hormônio do Crescimento/fisiopatologia , Adenoma Hipofisário Secretor de Hormônio do Crescimento/terapia , Terapia de Reposição Hormonal/efeitos adversos , Hospitais Públicos , Humanos , Hipofisectomia/efeitos adversos , Masculino , México/epidemiologia , Hipófise/efeitos dos fármacos , Hipófise/efeitos da radiação , Hipófise/cirurgia , Padrões de Prática Médica , Prevalência , Radioterapia/efeitos adversos , Sistema de Registros , Estudos Retrospectivos , Somatostatina/análogos & derivados , Somatostatina/uso terapêutico , Resultado do TratamentoRESUMO
BACKGROUND: Giant prolactinomas are an unusual subset of macroprolactinomas and are more commonly found in men. The goal of this review is to propose a giant prolactinoma definition and discuss the available therapeutic options for biochemical and tumour volume control. METHODS: A comprehensive search of all published studies was performed between April and November 2012 in electronic databases (PubMed and Ovid). RESULTS: A giant prolactinoma should be defined as an adenoma with a maximum diameter of more than 4 cm that is associated with serum prolactin above 5300 mIU/l. Regarding treatment, cabergoline is the preferred dopamine agonist for medical management of giant prolactinomas because of its excellent efficacy and tolerability. Normalization of prolactin level and significant tumour reduction may be achieved in the majority of patients. Combined therapy, particularly cabergoline and surgery, may be necessary due to the large tumour load. Radiotherapy and temozolomide may be used for patients with aggressive giant prolactinomas in whom tumour volume control is not achieved with cabergoline and surgery. CONCLUSION: There is a scarcity of large studies about the management of giant prolactinoma. Cabergoline is the first-line treatment. However, caution should be exercised when comparing efficacy rates among the different treatment modalities due to the variability in study design and data quality. In this scenario, a 'standard' definition for giant prolactinomas and larger series may be helpful to assess the real efficacy and safety of each therapeutic modality.
Assuntos
Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/terapia , Prolactinoma/patologia , Prolactinoma/terapia , Cabergolina , Terapia Combinada , Agonistas de Dopamina/uso terapêutico , Ergolinas/uso terapêutico , Humanos , Hipófise/efeitos dos fármacos , Hipófise/efeitos da radiação , Hipófise/cirurgia , Neoplasias Hipofisárias/sangue , Prolactina/sangue , Prolactinoma/sangue , Resultado do Tratamento , Carga TumoralRESUMO
BACKGROUND AND AIMS: Transsphenoidal surgery remains the treatment of choice in acromegaly, yet 40-50% of patients require secondary forms of therapy such as radiation therapy (RT) and somatostatin analogues (SA). We undertook this study to evaluate the efficacy and safety of RT in acromegaly. METHODS: Forty patients with acromegaly treated with RT (mean dose, 52 Gy) after failed pituitary surgery between 1993 and 2007 were analyzed; all were clinically and biochemically active. Patients were evaluated with yearly hormonal measurements [basal and glucose-suppressed growth hormone (GH), IGF-1, thyroid-stimulating hormone (TSH), free T4, cortisol, luteinizing hormone (LH), follicle-stimulating hormone (FSH), testosterone or estradiol and prolactin (PRL)] and with magnetic resonance imaging every 2 years. RESULTS: Mean age of patients was 52.9 ± 12.1 years and 85% were female. All subjects had been followed for 1 year, 75% for 3 years, 70% for 5 years and 35% for 10 years. The median basal GH level fell from a baseline of 8.8 ng/mL to 2.27 ng/mL at 5 years (p = 0.001) and to 1.88 ng/mL at 10 years (p = 0.001). A GH <1 ng/mL was achieved by 46% and 57% of the patients at 5 and 10 years of follow-up, respectively. The proportion of patients achieving a normal IGF-1 was 36% at 5 years and 43% at 10 years. Before RT, hypothyroidism, hypocortisolism and hypogonadism were present in 44%, 26% and 74% of patients, respectively. After 5 years of follow-up (n = 28), these figures increased to 51%, 41% and 79% and over a third of the group had panhypopituitarism. One patient developed optic neuritis and another patient was diagnosed with a meningioma 10 years after RT. No cerebrovascular events or deaths occurred. CONCLUSIONS: RT is an effective, low-cost and reasonably safe means of controlling acromegalic activity, particularly useful in parts of the world where SA are not readily available.
Assuntos
Acromegalia/radioterapia , Hipófise/efeitos da radiação , Resultado do Tratamento , Acromegalia/sangue , Acromegalia/tratamento farmacológico , Acromegalia/cirurgia , Adulto , Idoso , Feminino , Hormônio do Crescimento Humano/sangue , Humanos , Fator de Crescimento Insulin-Like I/metabolismo , Masculino , Pessoa de Meia-Idade , Hipófise/efeitos dos fármacos , Hipófise/cirurgia , Hormônios Hipofisários/sangue , Hormônios Hipofisários/deficiência , Somatostatina/análogos & derivados , Somatostatina/farmacologia , Somatostatina/uso terapêuticoRESUMO
BACKGROUND: Intracranial dissemination of pituitary adenomas is a rare event that does not equate malignancy. Most of the cases have been reported as metastases from pituitary carcinoma. A case of papillary pituitary carcinoma developed 12 years after radiotherapy for prolactin-secreting hormone pituitary adenoma is presented. CASE DESCRIPTION: A 37-year-old woman was admitted for the second time with neurologic disturbance and hypertension. A gadolinium-enhanced magnetic resonance scan of the brain demonstrated a 50-mm enhanced mass (absent on previous studies) on the lateral ventricle, involving the left temporal lobe. The patient underwent a craniotomy and biopsy of the lesion that was consistent with pituitary carcinoma; it was immunoreactive to follicle-stimulating hormone, adrenocorticotropic hormone, pituitary tumor-transforming gene, and epithelial cell adhesion molecule. Transmission electron microscopy analysis confirmed the secretory pituitary tumor diagnosis. CONCLUSIONS: The tumor was considered a primary pituitary papillary carcinoma. The clinical course indicated that this tumor was the seedling of a pituitary tumor, although it could be interpreted as metastases from a pituitary carcinoma.
Assuntos
Carcinoma Papilar/diagnóstico , Neoplasias do Ventrículo Cerebral/diagnóstico , Segunda Neoplasia Primária/diagnóstico , Neoplasias Hipofisárias/diagnóstico , Prolactinoma/diagnóstico , Radioterapia/efeitos adversos , Adulto , Biomarcadores Tumorais/metabolismo , Carcinoma Papilar/etiologia , Carcinoma Papilar/terapia , Neoplasias do Ventrículo Cerebral/etiologia , Neoplasias do Ventrículo Cerebral/terapia , Diagnóstico Diferencial , Progressão da Doença , Evolução Fatal , Feminino , Humanos , Lactotrofos/patologia , Lactotrofos/efeitos da radiação , Ventrículos Laterais/patologia , Invasividade Neoplásica , Neoplasia Residual , Segunda Neoplasia Primária/etiologia , Segunda Neoplasia Primária/terapia , Hipófise/metabolismo , Hipófise/patologia , Hipófise/efeitos da radiação , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/terapia , Prolactinoma/complicações , Prolactinoma/terapia , Tempo , Fatores de TempoRESUMO
Long term follow-up of patients submitted to treatment of parasellar tumours region is important for the detection of late therapeutic complications. In this study the authors conducted an evaluation of six patients with craniopharyngioma, one with germinoma, one with meningioma, and one epidermoid cyst. All above tumours were localized at parasellar region. Six out of nine patients had been treated both by surgery and by radiotherapy and the other three surgically only, on an average 3.8 +/- 3.2 years before this observation was carried out. Five patients were female with their ages average 24.3 +/- 18.8 years old. Evaluation consisted: in the first place, an intravenous infusion of thyrotropin-releasing hormone (TRH, 200 micrograms), gonadotropin-releasing hormone (GnRH, 100 micrograms), and insulin tolerance test (0.1 IU/Kg, regular insulin); and secondly, in measurements of pituitary hormones secretion at different time points--0, 20, 40, 60 and 80 minutes. We found both diminished response of growth hormone and cortisol in all the patients. Seven out of nine patients did not have adequate response to follicle-stimulating hormone. Three out of nine responded unsatisfactory to luteinizing hormone. Four out of nine showed inadequate responses to prolactin as well as, two out of eight to thyrotropin. We concluded that: (a) growth hormone and cortisol deficiency are the most frequent finding in these patients; (b) post-radiotherapy lesions can be located in the hypothalamus or pituitary, or even in both; (c) hypophysial and hypothalamic cells sensitivity to irradiation is different, according to their respective hormones; and (d) it is necessary a frequent endocrinologic follow-up of patients to detect late hormonal deficiencies.
Assuntos
Neoplasias Encefálicas/radioterapia , Hipotálamo/efeitos da radiação , Hipófise/efeitos da radiação , Hormônios Adeno-Hipofisários/sangue , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Hormônio Liberador de Gonadotropina , Hormônio do Crescimento/metabolismo , Humanos , Insulina , Masculino , Pessoa de Meia-Idade , Prolactina/metabolismo , Hormônio Liberador de TireotropinaRESUMO
O acompanhamento a longo prazo de pacientes submetidos a tratamento de tumores da regiäo parasselar é importante para detectar complicaçöes tardias da terapêutica. Neste estudo avaliamos 6 pacientes com craniofaringioma, 1 com meningioma, 1 com germinoma e 1 com cisto epidermóide, localizados na regiäo parasselar. Eles haviam sido tratados, em média, 3,8 ñ 3,2 anos antes, por cirurgia e radioterapia (6 casos) ou somente cirurgia (3 casos). Cinco pacientes eram do sexo feminino e a média de idade era de 24,3 ñ 18,8 anos. A avaliaçäo consistiu na infusäo endovenosa de TRH (200 µg), GnRH (100 µg) e insulina regular (0,1 UI Kg/peso), bem como na dosagem dos hormônios hipofisários antes (0) e após 20, 40, 60 e 80 minutos. Encontramos os seguintes resultados: (a) resposta deficitária do GH e do cortisol em todos pacientes; (b) 7/9 pacientes näo tiveram repostas adequadas do FSH e 3/9 do LH; (c) 4/9 tiveram respostas inadequadas da prolactina e 2/8 do TSH. Concluimos que: (a) o déficit de GH e cortisol säo os mais frequentes nestes pacientes; (b) a lesäo após radioterapia pode localizar-se tanto no hipotálamo quanto na hipóise ou, ainda, em ambos; (c) sa sensibilidade das células hipofisárias e hipotalâmicas à irradiaçäo é diferente de acordo com os hormônios que produzem; (d) é necessário o acompanhamento endocrinológico frequente dos pacientes submetidos a tratamento de tumores parasselares, a fim de detectar déficits hormonais tardios