Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 611
Filtrar
1.
Rev. neurol. (Ed. impr.) ; 71(5): 163-170, 1 sept., 2020. ilus, tab, graf
Artigo em Espanhol | IBECS | ID: ibc-195466

RESUMO

INTRODUCCIÓN: Los adenomas hipofisarios no funcionantes son el grupo tumoral más frecuente en la región selar. Suelen ser neoplasias benignas diagnosticadas por síntomas visuales u hormonales, aunque no es infrecuente detectarlos como un hallazgo casual. OBJETIVO: Analizar los aspectos clínicos hallados en esta enfermedad y su respuesta tras el tratamiento quirúrgico. PACIENTES Y MÉTODOS: En una serie de 100 casos, se analizaron datos epidemiológicos, clínicos, endocrinológicos, visuales y radiológicos antes y después del tratamiento quirúrgico, y se recogen las complicaciones relacionadas con la cirugía y el seguimiento a largo plazo. RESULTADOS: El síntoma más frecuente en el momento del diagnóstico fue la afectación del campo visual (62%), y sólo el 7% de los adenomas se trataba de un hallazgo casual. El déficit hormonal más frecuente era el hipogonadismo hipogonadótropo (48%). Tras la cirugía se observó recuperación completa del defecto campimétrico en el 54,8% de los pacientes, con sólo un 1% de empeoramiento tras la cirugía, y la incidencia de diabetes insípida fue del 4%. La resección fue superior al 95% en el 63% de los casos, a pesar de que el porcentaje de adenomas con invasión del seno cavernoso en grados altos fue elevado (45%). CONCLUSIONES: Aunque el síntoma más frecuente de los adenomas hipofisarios no funcionantes es la afectación campimétrica, ésta tiene una excelente respuesta a la cirugía si se realiza dentro del tiempo adecuado. El grado de invasión del seno cavernoso parece el factor más limitante para una resección quirúrgica completa


INTRODUCTION: Non-functioning pituitary adenomas are the most frequent tumor group in the sellar region. They are usually benign neoplasms diagnosed after visual or hormonal symptoms, although it is not uncommon to detect them as a casual finding. AIM: To analyze the clinical aspects found in this disease and its response after surgical treatment. PATIENTS AND METHODS: In a series of 100 cases, epidemiological, clinical, endocrinological, visual and radiological data were analyzed before and after surgical treatment, as well as the complications related to surgery and long-term follow-up. RESULTS: The most frequent symptom at the time of diagnosis was visual field involvement (62%), and only the 7% of adenomas were a casual finding. The most common hormonal deficit was hypogonadotropic hypogonadism (48%). After surgery, complete recovery of the visual field defect was observed in 54.8% of the patients, only 1% worsening after surgery, and the incidence of diabetes insipidus was 4%. The resection was superior to 95% in 63% of cases, although the percentage of adenomas with invasion of the cavernous sinus in Knosp grades 3 and 4 it was high (45%). CONCLUSIONS: Although the most frequent symptom of non-functioning pituitary adenomas is campimetric involvement, it has an excellent response to surgery if it is performed within the appropriate time. The grade of invasion of the cavernous sinus is the most limiting factor for a complete surgical resection


Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/cirurgia , Adenoma/cirurgia , Neoplasias Hipofisárias/diagnóstico por imagem , Adenoma/patologia , Complicações Pós-Operatórias/fisiopatologia , Complicações Pós-Operatórias/diagnóstico , Endoscopia , Hemianopsia , Seio Cavernoso/diagnóstico por imagem , Seio Cavernoso/cirurgia
2.
Eur J Endocrinol ; 183(4): 369-379, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32621582

RESUMO

Background: The '3PAs' syndrome, associating pituitary adenoma (PA) and pheochromocytoma/paraganglioma (PPGL), is sometimes associated with mutations in PPGL-predisposing genes, such as SDHx or MAX. In '3PAs' syndrome, PAs can occur before PPGL, suggesting a new gateway into SDHx/MAX-related diseases. Objective: To determine the SDHx/MAX mutation prevalence in patients with isolated PAs and characterize PAs of patients with SDHx/MAX mutations. Design: Genes involved in PAs (AIP/MEN1/CDKN1B) or PPGLs (SDHx/MAX) were sequenced in patients with isolated PAs. We then conducted a review of cases of PA in the setting of '3PAs' syndrome. Results: A total of 263 patients were recruited. Seven (likely) pathogenic variants were found in AIP, two in MEN1, two in SDHA, and one in SDHC. The prevalence of SDHx mutations reached 1.1% (3/263). Of 31 reported patients with PAs harboring SDHx/MAX mutations (28 published cases and 3 cases reported here), 6/31 (19%) developed PA before PPGL and 8/31 (26%) had isolated PA. The age of onset was later than in patients with AIP/MEN1 mutations. PAs were mainly macroprolactinomas and showed intracytoplasmic vacuoles seen on histopathology. Conclusions: We discovered SDHx mutations in patients bearing PA who had no familial or personal history of PPGL. However, the question of incidental association remains unresolved and data to determine the benefit of SDHx/MAX screening in these patients are lacking. We recommend that patients with isolated PA should be carefully examined for a family history of PPGLs. A family history of PPGL, as well as the presence of intracytoplasmic vacuoles in PA, requires SDHx/MAX genetic testing of patients.


Assuntos
Adenoma/genética , Mutação em Linhagem Germinativa , Neoplasias Hipofisárias/genética , Succinato Desidrogenase/genética , Adenoma/epidemiologia , Adenoma/patologia , Adolescente , Neoplasias das Glândulas Suprarrenais/epidemiologia , Neoplasias das Glândulas Suprarrenais/genética , Neoplasias das Glândulas Suprarrenais/patologia , Adulto , Idade de Início , Idoso , Fatores de Transcrição de Zíper de Leucina e Hélice-Alça-Hélix Básicos/genética , Criança , Análise Mutacional de DNA/métodos , Feminino , França/epidemiologia , Predisposição Genética para Doença , Testes Genéticos , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Isoenzimas/genética , Masculino , Pessoa de Meia-Idade , Paraganglioma/epidemiologia , Paraganglioma/genética , Paraganglioma/patologia , Feocromocitoma/epidemiologia , Feocromocitoma/genética , Feocromocitoma/patologia , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/patologia , Prolactinoma/epidemiologia , Prolactinoma/genética , Prolactinoma/patologia , Subunidades Proteicas/genética , Estudos Retrospectivos , Adulto Jovem
3.
Eur J Endocrinol ; 183(1): C1-C4, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32487776

RESUMO

Acromegaly is a debilitating and disfiguring chronic disease, which occurs in both sexes at any age, associated with multiple comorbidities and increased mortality. It is typically caused by a GH-secreting pituitary adenoma that promotes exposure of body tissues to increased concentrations of GH and IGF-I. The diagnosis of acromegaly is still made very late in a substantial number of patients when the disease is already in advanced stages. An epidemiological study from Sweden has elegantly demonstrated that the longer the diagnostic delay in acromegaly, the higher the number of comorbidities. Moreover, about 25% of the Swedish patients had 10 years or more of diagnostic delay and in this group mortality rate was significantly increased. These results reinforce the importance of shortening the latency period between disease onset, diagnosis and treatment to improve patient outcomes. This commentary article discusses strategies to be embraced by the endocrine community to allow early identification of acromegaly among public and health professionals, as internists, primary care clinicians, different specialists and dentists are the first point of contact for most of the patients. We emphasize that acromegaly should be presented as a sporadic, rather than rare, insidious disease, meaning that there is a considerable chance for health professionals to see a patient with acromegaly throughout their careers. The motto 'you must know it to think of it' is advocated in awareness efforts to reduce time to diagnosis, which results in lower rates of morbidity and mortality and might positively impact healthcare costs.


Assuntos
Acromegalia/diagnóstico , Acromegalia/epidemiologia , Neoplasias Hipofisárias/complicações , Acromegalia/etiologia , Diagnóstico Tardio , Feminino , Humanos , Masculino , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/epidemiologia
4.
Best Pract Res Clin Endocrinol Metab ; 34(2): 101418, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32414619

RESUMO

Cushing syndrome (CS) describes the signs and symptoms caused by exogenous or endogenous hypercortisolemia. Endogenous CS is caused by either ACTH-dependent sources (pituitary or ectopic) or ACTH-independent (adrenal) hypercortisolemia. Several genes are currently known to contribute to the pathogenesis of CS. Germline gene defects, such as MEN1, AIP, PRKAR1A and others, often present in patients with pituitary or adrenal involvement as part of a genetic syndrome. Somatic defects in genes, such as USP8, TP53, and others, are also involved in the development of pituitary or adrenal tumors in a large percentage of patients with CS, and give insight in pathways involved in pituitary or adrenal tumorigenesis.


Assuntos
Síndrome de Cushing/genética , Análise Mutacional de DNA , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/epidemiologia , Neoplasias das Glândulas Suprarrenais/genética , Carcinogênese/genética , Carcinogênese/patologia , Síndrome de Cushing/epidemiologia , Análise Mutacional de DNA/métodos , Análise Mutacional de DNA/tendências , Estudos de Associação Genética/métodos , Estudos de Associação Genética/tendências , Predisposição Genética para Doença , Mutação em Linhagem Germinativa/fisiologia , Humanos , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/genética , Transdução de Sinais/genética
5.
Best Pract Res Clin Endocrinol Metab ; 34(2): 101382, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32139169

RESUMO

Pituitary surgery is the first-line treatment for patients with Cushing's disease. For patients who are not considered candidates for pituitary surgery, pituitary radiation and bilateral adrenalectomy are further treatment alternatives. Not all patients are cured with pituitary surgery, and a substantial number of patients develop recurrence, sometimes many years after an apparently successful treatment. The same applies to patients treated with radiotherapy. Far from all patients are cured, and in many cases the disease recurs. Bilateral adrenalectomy, although always curative, causes chronic adrenal insufficiency and the remaining pituitary tumour can continue to grow and cause symptoms due to pressure on adjacent tissues, a phenomenon called Nelson's syndrome. In this paper the rate of recurrence of hypercortisolism, as well as the rate of development of Nelson's syndrome, following treatment of patients with Cushing's syndrome, will be reviewed. The aim of the paper is also to summarize clinical and biochemical factors that are associated with recurrence of hypercortisolism and how the patients should be monitored following treatment.


Assuntos
Biomarcadores/análise , Síndrome de Cushing/diagnóstico , Síndrome de Cushing/terapia , Diagnóstico por Imagem/métodos , Técnicas de Diagnóstico Endócrino , Monitorização Fisiológica/métodos , Doença de Addison/diagnóstico , Doença de Addison/epidemiologia , Doença de Addison/etiologia , Doença de Addison/terapia , Adenoma/complicações , Adenoma/diagnóstico , Adenoma/epidemiologia , Adenoma/terapia , Adrenalectomia/efeitos adversos , Síndrome de Cushing/epidemiologia , Síndrome de Cushing/patologia , Humanos , Síndrome de Nelson/diagnóstico , Síndrome de Nelson/epidemiologia , Síndrome de Nelson/etiologia , Síndrome de Nelson/cirurgia , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/terapia , Recidiva
6.
Rev Invest Clin ; 72(1): 8-18, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32132734

RESUMO

The pituitary gland is responsible for the synthesis and secretion of various hormones that play a key role in regulating endocrine function and homeostasis. Pituitary adenomas (PA) are benign epithelial tumors arising from the endocrine cells of the anterior pituitary gland. Clinically relevant PA are relatively common and they occur in 0.1% of the general population. They are mostly benign monoclonal neoplasms that arise from any of the five hormone-secreting cell types of the anterior pituitary gland. PA are categorized as either functioning or non-functioning, depending on whether or not they produce a hormonal hypersecretion syndrome. Both functioning and non-functioning adenomas can produce symptoms or signs resulting from compression of the optic chiasm or invasion of cavernous sinuses. Only 5% of PA occur within the context of hereditary syndromes with reasonably well-defined oncogenic mechanisms. The vast majority of PA are sporadic, and their etiopathogenesis remains largely unknown. Pituitary tumor oncogenesis involves several mechanisms that eventually lead to abnormal cell proliferation and dysregulated hormone production. Among these factors, we found inactivating mutations of tumor suppressor genes, activating mutation of oncogenes and the participation of hormonal signals coming from the hypothalamus, all resulting in cell-cycle regulation abnormalities. In this review, we summarize the clinical and pathophysiological aspects of the different hereditary pituitary tumor syndromes.


Assuntos
Adenoma/patologia , Hipófise/patologia , Neoplasias Hipofisárias/patologia , Adenoma/epidemiologia , Adenoma/genética , Animais , Humanos , Mutação , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/genética , Síndrome
8.
World Neurosurg ; 133: e211-e217, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31493596

RESUMO

BACKGROUND: Craniopharyngiomas show a high recurrence rate despite their pathologically benign nature. Thus, we analyzed the clinical features to elucidate the prognostic factors for the recurrence of craniopharyngiomas in adults with long-term follow-up. METHODS: This retrospective study reviewed and analyzed the preoperative features, surgical results, and tumor recurrence of patients who underwent an operation at a single institution from 2004 to 2013. RESULTS: This study analyzed the results of 64 consecutive adult patients, and the median follow-up period was 83.5 months (range 9-163 months). Ten patients had a history of surgery, whereas 4 had a history of adjuvant radiation. Retrochiasmatic tumors (n = 51, 79.7%) were more common than prechiasmatic tumors. Operations were performed via the transcranial approach in 31 (48.4%) patients and transsphenoidal approach in 33. Gross total removal was achieved in 44 (68.8%) patients, and the transsphenoidal approach showed a greater gross total removal rate than the transcranial approach did (97.0% vs. 38.7%, P < 0.001). Adjuvant radiotherapy was performed in 8 patients, and radiosurgery was performed in 2. Recurrence was identified in 25 (39.1%) patients in 45.0 months of the median time to recurrence. The overall actuarial 5- and 7-year progression-free survival rates were 71.8% and 63.6%, respectively. Multivariate analysis revealed that supra- and subdiaphragmatic tumor locations and subtotal removal were risk factors for long-term tumor recurrence. CONCLUSIONS: The long-term recurrence rate of craniopharyngiomas was high; therefore, long-term regular follow-up is mandatory. Adjuvant irradiation for remnant tumors showed a long-term tumor control rate comparable to that of completely removed tumors.


Assuntos
Craniofaringioma/epidemiologia , Recidiva Local de Neoplasia/epidemiologia , Neoplasias Hipofisárias/epidemiologia , Adulto , Idoso , Terapia Combinada , Craniofaringioma/complicações , Craniofaringioma/radioterapia , Craniofaringioma/cirurgia , Feminino , Seguimentos , Humanos , Hidrocefalia/etiologia , Hipofisectomia/métodos , Hipopituitarismo/etiologia , Masculino , Pessoa de Meia-Idade , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/radioterapia , Neoplasias Hipofisárias/cirurgia , Complicações Pós-Operatórias , Prognóstico , Intervalo Livre de Progressão , Radiocirurgia , Radioterapia Adjuvante , Estudos Retrospectivos , Transtornos da Visão/etiologia
9.
J Clin Endocrinol Metab ; 105(1)2020 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-31589293

RESUMO

CONTEXT: Craniopharyngioma is a rare neoplastic entity of the central nervous system. Childhood-onset craniopharyngioma is the subject of frequent research whereas the information on adult-onset craniopharyngioma is scarce. OBJECTIVE: The objective of this study was to examine the level of daily impairment in adult patients suffering from craniopharyngioma. DESIGN: Noninterventional patient registry indexed as PV4842 with the local ethics committee. SETTING: The study is set in a hospitalized and ambulatory setting. PATIENTS: 148 patients with adult-onset craniopharyngioma were recruited from 8 centers, 22 prospectively and 126 retrospectively. Mean follow-up was 31 months. INTERVENTIONS: No interventions performed. MAIN OUTCOME MEASURES: Complications, symptoms, body mass index (BMI), and quality of life (QoL; EORTC QLQ C30 and BN20) were recorded preoperatively and at follow-up. The hypotheses tested were generated after data collection. RESULTS: Complications were more frequent after transcranial than transsphenoidal approaches (31 % vs. 11%; P < 0.01). Preoperative obesity was present in 0% papillary and in 38% of all adamantinomatous craniopharyngiomas (P = 0.05), and diabetes insipidus was more frequent for papillary craniopharyngioma (36.8% vs. 16,7%; P < 0.05). Hormone deficits at follow-up were reduced in 16.9%, equal in 31.4%, and increased in 63.6% (P < 0.001). BMI increased from 28.7 ± 7.4 kg/m2 before surgery to 30.2 ± 7.4 kg/m2 at follow-up (P < 0.001). In QoL, a decrease of future uncertainty (62.5 vs. 36.8; P = 0.02) and visual disorders (38.9 vs. 12.0; P = 0.01) were observed in the prospective collective after surgery. CONCLUSIONS: Adult craniopharyngioma is associated with a complex sociological and psychological burden and hypothalamic dysfunction, warranting further investigation and emphasizing the need for a wider treatment approach.


Assuntos
Craniofaringioma , Neoplasias Hipofisárias , Adolescente , Adulto , Idade de Início , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Craniofaringioma/diagnóstico , Craniofaringioma/epidemiologia , Craniofaringioma/terapia , Feminino , Seguimentos , Alemanha/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos/efeitos adversos , Procedimentos Neurocirúrgicos/estatística & dados numéricos , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/terapia , Complicações Pós-Operatórias/epidemiologia , Qualidade de Vida , Estudos Retrospectivos , Osso Esfenoide/cirurgia , Adulto Jovem
10.
Pituitary ; 23(1): 3-8, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31641970

RESUMO

Lactotroph adenomas, also called prolactinomas and prolactin-secreting adenomas, constitute nearly 80% of functioning pituitary tumors and about 30-50% of all adenomas in the clinical practice. Lactotroph adenomas occur in the general population at a prevalence of 45/100,000, are more common in women, but also involve men and children of both sexes. Most lactotroph adenomas are microadenomas occurring in reproductive-age women who present with oligo/amenorrhea, galactorrhea, and infertility. In men and elderly women, lactotroph adenomas are usually macroadenomas and are most commonly associated with symptoms of a tumoral mass, including headaches, neurologic defects, and visual loss. Although clinical and laboratory features may differ depending on patient's gender and age, the histopathology of the tumors is similar. Lactotroph adenomas are histologically classified into three subtypes: the common sparsely granulated lactotroph adenoma, and the rare densely granulated lactotroph adenoma and acidophilic stem cell adenoma. We will review the main pathological features of the lactotroph adenomas and some of their characteristics that may predict biological behavior and responsiveness to treatment.


Assuntos
Prolactinoma/epidemiologia , Feminino , Humanos , Masculino , Neoplasias Hipofisárias/epidemiologia , Prevalência
11.
PLoS One ; 14(12): e0226033, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31830115

RESUMO

INTRODUCTION: Cushing's disease (CD) is a rare cause of hypercortisolemia presenting a major diagnostic and therapeutic challenge. Data on pituitary function in long-term follow-up after CD treatment in childhood is limited. AIM: Long-term assessment of patients of the Children's Memorial Health Institute (CMHI) after CD treatment in childhood. MATERIALS AND METHODS: Retrospective analysis of 29 CD patients, mean age at the time of diagnosis 13.46 yrs. The long-term follow-up (FU) was done by: 1) obtaining the data from a patient's questionnaire (75% of adult patients); 2) using the data from the last clinic visit for patients who did not respond to the questionnaire and for current CMHI patients. The average long-term FU from transsphenoidal pituitary surgery (TSS) was 10.23 yrs. RESULTS: At the latest FU: 18 patients (62%) had long-term disease remission after TSS1, 2 patients (6.9%) after TSS2, 1 patient (3.4%) after the post-TSS radiotherapy (XRT) cycle and 3 patients (10.3%) after bilateral adrenalectomy (BA). One patient (3.4%) died after TSS2 due to postoperative complications, 1 patient (3.4%) had persistent disease at latest FU, in 1 patient (3.4%) the long-term FU was not possible to perform. CD recurrence occurred in 4 out of 28 patients (14%) at an average time 3.6 yrs. from definitive treatment. One patient (3.4%) after BA was operated because of Nelson's syndrome. Two patients (6.9%) were suspected of relapse at latest assessment. At the time of the last evaluation, 17 patients (63%) were on levothyroxine therapy since definitive treatment, 16 patients (59%) were on hydrocortisone treatment, 10 patients (37%) were taking sex hormones replacement, 4 patients (15%)-antidiuretic hormone. CONCLUSIONS: Relatively large number of patients after CD treatment in childhood have hormonal pituitary deficits as well as mood and cognitive disorders. CD recurrence can occur even after a long time post effective treatment.


Assuntos
Hipersecreção Hipofisária de ACTH/epidemiologia , Hipersecreção Hipofisária de ACTH/terapia , Adenoma/complicações , Adenoma/epidemiologia , Adenoma/metabolismo , Adenoma/terapia , Adolescente , Adulto , Idade de Início , Criança , Desenvolvimento Infantil/fisiologia , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Recidiva Local de Neoplasia/epidemiologia , Hipersecreção Hipofisária de ACTH/fisiopatologia , Testes de Função Hipofisária , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/metabolismo , Neoplasias Hipofisárias/terapia , Polônia/epidemiologia , Puberdade/fisiologia , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
12.
Ann Endocrinol (Paris) ; 80 Suppl 1: S19-S28, 2019 Sep.
Artigo em Francês | MEDLINE | ID: mdl-31606058

RESUMO

Multiple Endocrine Neoplasia Type 1 (NEM1) is related to mutations of the menin gene. It is an autosomal dominant disease. Its prevalence is about 1/30 000 with a hugh penetrance. There is no genotype-phenotype correlation. This hereditary syndrome is characterized by the presence of tumors of the endocrine system (parathyroid, endocrine pancreas, pituitary and adrenal gland). Other disorders have also been described (bronchial and thymic carcinoid tumor, breast cancer, skin lesions). Management must take into account the specificities of these pathologies in NEM1 compared to sporadic forms (young age at diagnosis, multiple lesions within the same gland, multi-focal disease). © 2019 Published by Elsevier Masson SAS. All rights reserved. Cet article fait partie du numéro supplément Les Must de l'Endocrinologie 2019 réalisé avec le soutien institutionnel de Ipsen-Pharma.


Assuntos
Congressos como Assunto , Endocrinologia/tendências , Neoplasia Endócrina Múltipla Tipo 1 , Tumor Carcinoide/diagnóstico , Tumor Carcinoide/epidemiologia , Tumor Carcinoide/genética , Tumor Carcinoide/terapia , Congressos como Assunto/organização & administração , Congressos como Assunto/tendências , Endocrinologia/métodos , Endocrinologia/organização & administração , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/epidemiologia , Neoplasias Gastrointestinais/genética , Neoplasias Gastrointestinais/terapia , Humanos , Neoplasia Endócrina Múltipla Tipo 1/diagnóstico , Neoplasia Endócrina Múltipla Tipo 1/epidemiologia , Neoplasia Endócrina Múltipla Tipo 1/genética , Neoplasia Endócrina Múltipla Tipo 1/terapia , Mutação , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/epidemiologia , Tumores Neuroendócrinos/genética , Tumores Neuroendócrinos/terapia , Penetrância , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/genética , Neoplasias Hipofisárias/terapia , Proteínas Proto-Oncogênicas/genética , Sociedades Médicas/organização & administração , Sociedades Médicas/normas , Sociedades Médicas/tendências
13.
Eur J Endocrinol ; 181(5): R185-R197, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31416048

RESUMO

Visual dysfunction is an important element in the morbidity encountered in patients with pituitary adenoma leading to functional impairment and compromised quality of life. It consists of many parameters (even in the absence of reported symptomatology) as a result of tumour growth in proximity to structures critical for vision (anterior visual pathway, cranial nerves within cavernous sinuses), and as an adverse consequence of therapeutic interventions. Adenoma resection leads to high rates of visual improvement and possibly continues beyond a year post surgery, but the exact timing of maximum effect requires elucidation. Retinal nerve fibre layer measurement may be a reliable, objective parameter predicting favourable visual outcomes, although its prognostic value when pathological, needs to be confirmed. For compromised vision after pituitary apoplexy, early surgical decompression remains usual practice until evidence-based guidance becomes available. The risk of radiation-induced visual toxicity is mainly influenced by total and per fraction dose of radiation and treatment modality. Careful selection of cases and of radiotherapy technique/planning are of major importance in minimising this risk. Dopamine agonists lead to visual recovery in a considerable number of prolactinoma patients. Visual morbidity should be considered a vital indicator in the metrics of quality of service/care in pituitary disease making regular, full ophthalmic examination an essential component of modern management of pituitary pathology at all time points of patient pathway. Well-designed studies minimising effects of bias and using tools and scoring systems reliably reflecting visual status will provide robust evidence on valid prognostication and patient stratification guiding clinical decision making.


Assuntos
Adenoma/complicações , Adenoma/epidemiologia , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/epidemiologia , Transtornos da Visão/epidemiologia , Transtornos da Visão/etiologia , Humanos , Planejamento de Assistência ao Paciente , Transtornos da Visão/patologia
14.
Best Pract Res Clin Endocrinol Metab ; 33(2): 101309, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-31405752

RESUMO

Pituitary diseases are rare conditions with severe chronic multiorgan and multisystemic morbidity requiring complex multidisciplinary treatment and usually life-long drug treatment. Most cases are caused by functioning or non-functioning pituitary adenoma. From the patient's perspective, the burden of disease is caused by the tumour itself and associated compression symptoms, interventions, hormone excess and deficiencies, systemic manifestations of these endocrine abnormalities and general psychosocial issues that can manifest in patients with a chronic condition. In this review, patient burden is classified according to classic endocrine syndromes, with burden at diagnosis and after long-term remission, and also within the framework of value-based health care and the conceptual model of wellbeing. The recently developed patient-reported outcome measurement tool that helps to evaluate burden of patients is also discussed.


Assuntos
Adenoma , Efeitos Psicossociais da Doença , Neoplasias Hipofisárias , Acromegalia/epidemiologia , Acromegalia/etiologia , Acromegalia/psicologia , Acromegalia/terapia , Adenoma/diagnóstico , Adenoma/epidemiologia , Adenoma/psicologia , Adenoma/terapia , Humanos , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/psicologia , Neoplasias Hipofisárias/terapia , Qualidade de Vida
15.
Radiat Oncol ; 14(1): 152, 2019 Aug 27.
Artigo em Inglês | MEDLINE | ID: mdl-31455428

RESUMO

AIM: To quantify the prevalence of brain metastases involving the hypothalamic-pituitary (HT-P) area. INTRODUCTION: Cognitive impairment and fatigue are common side effects of whole brain irradiation (WBI) comprising the quality of life (QoL) for survivors. While the former is related to radiation-induced hippocampal injury, the latter could be secondary to hormonal disbalance as a consequence of radiation of the HT-P area. Thus, sparing both regions from higher irradiation doses could reduce these sequelae. METHODS: T1 contrast medium enhanced magnetic resonance imaging (MRI) scans of 865 patients with brain metastases (4,280 metastases) were reviewed. HT-P area was individually contoured with a margin of 5 mm in order to evaluate the prevalence of brain metastases in this region. RESULTS: Involvement of the hypothalamic region was found in 26 patients (involvement rate of 3% for patients and 1% for metastases), involvement of the pituitary gland in 9 patients (1% for patients and < 1% for metastases). Binary logistical regression analysis revealed the presence of > 10 brain metastases as the only factor associated with hypothalamic involvement while no distinct factor was associated with an involvement of the pituitary gland. CONCLUSION: The low prevalence of metastases within the HT-P area in patients with brain metastases calls for further studies examining whether sparing of this region might improve patients QoL.


Assuntos
Neoplasias Encefálicas/radioterapia , Irradiação Craniana/efeitos adversos , Hipocampo/patologia , Neoplasias/radioterapia , Neoplasias Hipofisárias/epidemiologia , Lesões por Radiação/etiologia , Planejamento da Radioterapia Assistida por Computador/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Encefálicas/secundário , Sobreviventes de Câncer/estatística & dados numéricos , Feminino , Alemanha/epidemiologia , Hipocampo/efeitos da radiação , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neoplasias/patologia , Tratamentos com Preservação do Órgão , Órgãos em Risco/efeitos da radiação , Neoplasias Hipofisárias/secundário , Prevalência , Prognóstico , Qualidade de Vida , Lesões por Radiação/patologia , Dosagem Radioterapêutica , Radioterapia de Intensidade Modulada/métodos , Taxa de Sobrevida
16.
Best Pract Res Clin Endocrinol Metab ; 33(2): 101299, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-31431397

RESUMO

In this paper, the currently used and well evaluated techniques for the surgery of pituitary tumors will be reviewed. Since the first surgical approaches to pituitary tumors more than 100 years have elapsed. Various surgical techniques have been developed, refined and standardized. Most of these tumors are to date treated via transsphenoidal approaches. Many pituitary adenomas, particularly, smaller, enclosed ones, can be completely excised and a selective adenomectomy is usually attempted. It leads to remission of hormonal oversecretion and also to recovery of pituitary function in many patients. The resection of pseudocapsule around the adenoma seems to improve the operative results further. Transcranial approaches, employing craniotomies, are still needed in some patients with pituitary adenomas and in many of those harbouring craniopharyngiomas. The operative techniques will be described and briefly commented. Moreover, the application and usefulness of several technical developments will be reviewed, such as the use of the endoscope, magnetic resonance imaging, fluorenscent dyes and neuronavigation. The use of the intraoperative Doppler probe, ultrasound and the value of intraoperative hormonal measurements will be briefly discussed. There is sufficient evidence that the best and optimal outcome in terms of tumor resection and correction of hormonal oversecretion as well as the lowest rate of complications are obtained in centers of excellence with sufficiently experienced, specialized surgeons and a high patient load.


Assuntos
Adenoma/cirurgia , Procedimentos Neurocirúrgicos/métodos , Neoplasias Hipofisárias/cirurgia , Adenoma/diagnóstico , Adenoma/epidemiologia , Humanos , Imagem por Ressonância Magnética , Microcirurgia/métodos , Procedimentos Neurocirúrgicos/normas , Hipófise/cirurgia , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/epidemiologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Resultado do Tratamento
17.
Hormones (Athens) ; 18(3): 251-254, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31388898

RESUMO

Double and multiple adenomas of the pituitary are composed of two or more distinct tumors located in the same gland. They represent uncommon lesions measuring less than 1 cm, reported as having a low incidence in autopsies and occurring even more infrequently in surgical series. The histological diagnosis of double adenomas in surgical material is often extremely difficult, and confirmation requires immunohistochemistry and, occasionally, electron microscopy. Fragmented tissue material submitted for histology after transsphenoidal resection complicates the diagnosis. Difficulties in demonstrating double or multiple adenomas by imaging techniques contribute to diagnostic failure. Magnetic resonance imaging (MRI) techniques may disclose two separate adenomas located in the same pituitary gland. Intraoperative MRI and imaging ultrasonography, together with positron emission computed tomography, more accurately identify sites of residual tumors. These techniques might also detect postoperatively a residual tumor belonging to the second component of double adenoma. Double adenomas may also create extreme clinical diagnostic challenges. It is almost impossible to suspect functioning double adenomas with combined hormone secretion, each one secreting a different hormone, and distinguish them from an isolated plurihormonal adenoma, simultaneously secreting more than one hormone. Double adenomas may underlie surgical failure when one adenoma is removed while the other is left behind. Despite the low frequency of double adenomas, identification and resection of both of them is of major importance for the achievement of biochemical cure.


Assuntos
Adenoma/diagnóstico , Técnicas de Diagnóstico Endócrino , Neoplasias Primárias Múltiplas/diagnóstico , Neoplasias Hipofisárias/diagnóstico , Adenoma/epidemiologia , Adenoma/patologia , Adenoma/terapia , Diagnóstico Diferencial , Técnicas de Diagnóstico Endócrino/normas , Humanos , Imagem por Ressonância Magnética , Neoplasia Residual , Neoplasias Primárias Múltiplas/epidemiologia , Neoplasias Primárias Múltiplas/patologia , Neoplasias Primárias Múltiplas/terapia , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/terapia , Tomografia por Emissão de Pósitrons , Prognóstico
18.
Endocrinol Metab Clin North Am ; 48(3): 569-581, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31345524

RESUMO

Pituitary adenomas are common. The impact of pituitary tumors on fertility are mainly caused by oversecretion and/or undersecretion of pituitary hormones or compression of pituitary stalk and normal pituitary tissue by the tumor. Diagnosing and managing pituitary tumors during pregnancy involve many challenges, including the effect of hormone excess or deficiency on pregnancy outcome, changes in the pituitary or pituitary-related hormones, changes in tumor size, and the impact of various treatments of pituitary tumors on maternal and fetal outcomes. This article discusses the diagnosis and treatment of patients with prolactinomas, acromegaly, Cushing disease, and other pituitary tumors during pregnancy.


Assuntos
Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/terapia , Prolactinoma/diagnóstico , Prolactinoma/terapia , Acromegalia/diagnóstico , Acromegalia/epidemiologia , Acromegalia/etiologia , Acromegalia/terapia , Feminino , Humanos , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/epidemiologia , Gravidez , Prolactinoma/complicações , Prolactinoma/epidemiologia
19.
Best Pract Res Clin Endocrinol Metab ; 33(2): 101290, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-31326373

RESUMO

Prevalence and incidence of prolactinomas are approximately 50 per 100,000 and 3-5 new cases/100,000/year. The pathophysiological mechanism of hyperprolactinemia-induced gonadotropic failure involves kisspeptin neurons. Prolactinomas in males are larger, more invasive and less sensitive to dopamine agonists (DAs). Macroprolactin, responsible for pseudohyperprolactinemia is a frequent pitfall of prolactin assay. DAs still represent the primary therapy for most prolactinomas, but neurosurgery has regained interest, due to progress in surgical techniques and a high success rate in microprolactinoma, as well as to some underestimated side effects of long-term DA treatment, such as impulse control disorders or impaired quality of life. Recent data show that the suspected effects of DAs on cardiac valves in patients with prolactinomas are reassuring. Finally, temozolomide has emerged as a valuable treatment for rare cases of aggressive and malignant prolactinomas that do not respond to all other conventional treatments.


Assuntos
Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/terapia , Prolactinoma/diagnóstico , Prolactinoma/epidemiologia , Prolactinoma/terapia , Agonistas de Dopamina/uso terapêutico , Feminino , Humanos , Hiperprolactinemia/diagnóstico , Hiperprolactinemia/epidemiologia , Hiperprolactinemia/etiologia , Hiperprolactinemia/terapia , Incidência , Kisspeptinas/metabolismo , Masculino , Neurônios/metabolismo , Neurônios/patologia , Neoplasias Hipofisárias/complicações , Prevalência , Prolactina/análise , Prolactina/sangue , Prolactinoma/complicações , Qualidade de Vida
20.
Pediatr Radiol ; 49(13): 1781-1787, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31352514

RESUMO

BACKGROUND: Pituitary pars intermedia/Rathke cleft cysts or cyst-like structures are commonly encountered in children undergoing brain magnetic resonance imaging (MRI), especially when examinations include thin-section, high-resolution sequences. OBJECTIVE: To determine the prevalence of pituitary cystic lesions in children at our institution using modern MRI technique, to assess for associated endocrinopathy and to address the need for follow-up. MATERIALS AND METHODS: We retrospectively reviewed 232 consecutive 1.5- and 3-T brain MRIs in children ages 0-18 years (mean: 8.3±5.3 years). We evaluated 3-D volumetric T1 spoiled gradient echo (SPGR) and axial T2-weighted sequences. Pituitary glands were evaluated for the presence, size and signal characteristics of cysts. Cyst volumes were measured in three orthogonal planes. Endocrine abnormalities were documented from the medical record. RESULTS: Pituitary cysts were present in 57.7% of children (n=134), with a mean linear measurement of 3.6±1.17 mm (range: 0.4 to 12.3 mm). The overwhelming majority of cysts were hyopointense on T1-W images (n=121, 90%) and isointense on T2-W images relative to the adenohypophysis (n=106, 79%). T1 hyperintense and T2 hypointense signals were present in a minority, 6.7% and 8%, respectively. Most cysts were occult on post-contrast T1-W images (n=24, 77%). Endocrine abnormalities were present in 2/134 (1.5%) of children with cysts (these were unrelated to the pituitary gland) versus 1/98 (1%) children without cysts (P=0.7). CONCLUSION: More often than not, pituitary cysts/cyst-like structures can be found incidentally in children using modern MRI techniques. Follow-up is not typically required if there are no associated symptoms or excessive size.


Assuntos
Cistos do Sistema Nervoso Central/diagnóstico por imagem , Imageamento Tridimensional , Imagem por Ressonância Magnética/métodos , Neoplasias Hipofisárias/diagnóstico por imagem , Intensificação de Imagem Radiográfica , Adolescente , Distribuição por Idade , Cistos do Sistema Nervoso Central/epidemiologia , Cistos do Sistema Nervoso Central/patologia , Criança , Pré-Escolar , Estudos de Coortes , Meios de Contraste , Cistos , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/patologia , Prevalência , Estudos Retrospectivos , Medição de Risco
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA