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2.
BMJ Case Rep ; 14(1)2021 Jan 18.
Artigo em Inglês | MEDLINE | ID: mdl-33462053

RESUMO

An 85-year-old man was referred to endocrinology following the discovery of an incidental pituitary mass on cranial imaging which was thought to be a non-functioning adenoma during an admission with headaches, lethargy, confusion and hyponatraemia. He had a history of Hürthle cell carcinoma of the thyroid treated with total thyroidectomy, ablative radioiodine therapy and thyroxine replacement. Subsequently, he developed metastatic spread to the neck, lungs and skeleton. About 9 months later, the patient had deterioration of vision. MRI showed a rapidly expanding pituitary mass with compression of the optic chiasm. Biochemical investigations confirmed hypocortisolism and hypogonadism. The patient underwent trans-sphenoidal resection of the pituitary mass followed by external beam radiotherapy to the pituitary bed. Histopathology confirmed a metastatic deposit of Hürthle cell carcinoma, which is a rare and aggressive variant of follicular thyroid carcinoma.


Assuntos
Adenoma Oxífilo/diagnóstico , Adenoma Oxífilo/secundário , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/secundário , Neoplasias da Glândula Tireoide/diagnóstico , Neoplasias da Glândula Tireoide/secundário , Adenoma Oxífilo/terapia , Idoso de 80 Anos ou mais , Humanos , Masculino , Neoplasias Hipofisárias/terapia , Neoplasias da Glândula Tireoide/terapia
3.
Endocrinol. diabetes nutr. (Ed. impr.) ; 67(7): 469-485, ago.-sept. 2020. ilus, tab
Artigo em Inglês | IBECS | ID: ibc-194704

RESUMO

The concept of aggressive pituitary tumor (APT) has been precisely defined in recent years. These tumors are characterized by morphological (radiological or histopathological) data of invasion, proliferative activity superior to that of typical adenomas and a clinical behavior characterized by resistance to standard therapies and frequent recurrences. The absence of cerebrospinal or distant metastases differentiates them from the pituitary carcinoma. APTs account for about 10% of all pituitary neoplasm. Proper diagnostic implies participation not only of radiological and hormonal investigation but also a thorough pathological assessment including proliferation markers and immunohistochemistry for hormones and transcription factors. Surgical resection, aiming gross total resection or tumor debulking, is the mainstay initial therapy in most patients. Most patients with APTs need more than one surgical intervention, pituitary radiation, sometimes on more than one occasion, and multiple sequential or combined medical treatments, to finally be doomed to unusual treatments, such as alkylating agents (temozolomide alone or in combination), molecular targeted therapies, or peptide receptor radionuclide therapy. Multimodal therapy, implemented by experts, preferably in specialized centers with high volume caseload, is the only way to improve the prognosis of patients with these uncommon tumors. The research needs in this area are multiple and include a greater knowledge of the molecular biology of these tumors, establishment of protocols for monitoring and sequencing of treatments, development of multicenter studies and international registries


El concepto de tumor hipofisario agresivo (THA) se ha definido con más precisión en los últimos años. Son tumores caracterizados por signos morfológicos (radiológicos o histopatológicos) de invasión, actividad proliferativa superior a la de los adenomas típicos y un comportamiento clínico caracterizado por resistencia a los tratamientos habituales y recidivas frecuentes. La ausencia de metástasis cefalorraquídeas o a distancia los diferencia del carcinoma hipofisario. Los THA suponen alrededor del 10% de todas las neoplasias hipofisarias. Un diagnóstico apropiado exige no solo investigación radiológica y hormonal, sino también una valoración histopatológica detenida que incluya marcadores de proliferación e inmunohistoquímica para hormonas y factores de transcripción. La resección quirúrgica encaminada a la resección total o la reducción del volumen tumoral es el tratamiento inicial clave en la mayoría de los pacientes. La mayoría de los pacientes con THA necesitan más de una intervención quirúrgica, irradiación hipofisaria, a veces en más de una ocasión, y diversos tratamientos médicos consecutivos o combinados, y están predestinados a terminar recibiendo tratamiento inhabituales como fármacos alquilantes (temozolomida sola o en combinación), tratamientos multidiana o tratamientos con péptidos radiomarcados. El tratamiento multimodal aplicado por expertos, preferiblemente en centros especializados con gran volume de pacientes, es el único modo de mejorar el pronóstico de los pacientes con estos tumores poco frecuentes. Las necesidades de investigación en este campo son enormes, e incluyen la de un mayor conocimiento de la biología molecular de estos tumores, el establecimiento de protocolos de vigilancia y secuenciación de los tratamientos, el desarrollo de estudios multicéntricos y registros internacionales


Assuntos
Humanos , Neoplasias Hipofisárias/terapia , Terapia Combinada/métodos , Adenoma/terapia , Adenoma Hipofisário Secretor de Hormônio do Crescimento/terapia , Adenocarcinoma/terapia , Imuno-Histoquímica , Prolactinoma/terapia , Neurocirurgia , Biomarcadores , Alquilantes/uso terapêutico , Melanoma/epidemiologia
4.
Eur J Endocrinol ; 183(1): G17-G23, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32369770

RESUMO

Patients with pituitary tumours, ensuing hormonal abnormalities and mass effects are usually followed in multidisciplinary pituitary clinics and can represent a management challenge even during the times of non-pandemic. The COVID-19 pandemic has put on hold routine medical care for hundreds of millions of patients around the globe, while many pituitary patients' evaluations cannot be delayed for too long. Furthermore, the majority of patients with pituitary tumours have co-morbidities potentially impacting the course and management of COVID-19 (e.g. hypopituitarism, diabetes mellitus, hypertension, obesity and cardiovascular disease). Here, we summarize some of the diagnostic and management dilemmas encountered, and provide guidance on safe and as effective as possible delivery of care in the COVID-19 era. We also attempt to address how pituitary services should be remodelled in the event of similar crises, while maintaining or even improving patient outcomes. Regular review of these recommendations and further adjustments are needed, depending on the evolution of the COVID-19 pandemic status. We consider that the utilization of successful models of pituitary multidisciplinary care implemented during the COVID-19 pandemic should continue after the crisis is over by using the valuable and exceptional experience gained during these challenging times.


Assuntos
Adenoma/terapia , Antineoplásicos Hormonais/uso terapêutico , Infecções por Coronavirus , Agonistas de Dopamina/uso terapêutico , Procedimentos Neurocirúrgicos , Pandemias , Apoplexia Hipofisária/terapia , Neoplasias Hipofisárias/terapia , Pneumonia Viral , Adenoma/diagnóstico , Cabergolina/uso terapêutico , Gerenciamento Clínico , Terapia de Reposição Hormonal , Hormônio do Crescimento Humano/análogos & derivados , Hormônio do Crescimento Humano/uso terapêutico , Humanos , Octreotida/uso terapêutico , Peptídeos Cíclicos/uso terapêutico , Apoplexia Hipofisária/diagnóstico , Neoplasias Hipofisárias/diagnóstico , Guias de Prática Clínica como Assunto , Radioterapia , Somatostatina/análogos & derivados , Somatostatina/uso terapêutico , Telemedicina , Fatores de Tempo , Testes de Campo Visual
5.
J Clin Neurosci ; 75: 122-127, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32199742

RESUMO

Neurocognitive complaints are common in patients with pituitary tumours, particularly in memory and concentration. Past studies have shown impairments in executive function and memory, but it is not clear whether these result from direct effects of the tumour (pressure or hormonal secretion), incidental damage from radiotherapy or surgical treatments, and/or mediating psychiatric factors. This study assessed cognitive function and psychiatric state of 86 pituitary tumour patients and 18 healthy controls, pre and post-treatment, to examine the effects of tumour aetiology and treatment type. No significant cognitive impairments were found, except on verbal recognition memory. Patients with Cushing's disease showed lower verbal recognition memory than the other groups pre-treatment, but improved at follow-up. This was (at least partially) accounted for by an improvement in depression scores. Patients who were treated with surgery showed poorer verbal recognition memory than controls across all (pre- and post-treatment) time-points. Overall findings of minimal cognitive impairment in patients with pituitary tumours may reflect improved diagnostic and treatment techniques in recent years. We suggest that the verbal memory impairments identified in the Cushing's group may result from increased cortisol (directly, or mediated by depression). In the surgical groups, verbal memory impairments appeared to pre-date treatment. This may relate to treatment selection factors, rather than harmful effects of surgery itself.


Assuntos
Cognição , Neoplasias Hipofisárias/psicologia , Neoplasias Hipofisárias/terapia , Adulto , Atenção , Estudos de Casos e Controles , Transtornos Cognitivos/etiologia , Depressão , Feminino , Humanos , Masculino , Memória , Transtornos da Memória/etiologia , Pessoa de Meia-Idade , Hipersecreção Hipofisária de ACTH/psicologia , Neoplasias Hipofisárias/radioterapia , Neoplasias Hipofisárias/cirurgia , Estudos Prospectivos
6.
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
7.
Arq Neuropsiquiatr ; 78(1): 28-33, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-32074187

RESUMO

METHODS: Prolactin (PRL) secreting adenomas are associated with high incidence of headache. The role of hyperprolactinemia in the headache context is not clear, nor is the effect of its treatment on headache. The present longitudinal study evaluated hyperprolactinemic patients (69), in terms of presence and characteristics of headache before and after hyperprolactinemia treatment. RESULTS: Headache was reported by 45 (65.2%) patients, independent of the etiology of hyperprolactinemia. The migraine phenotype was the most prevalent (66.6%). Medications used in the treatment of headache not changed during the study. The first line of treatment of hyperprolactinemia was dopaminergic agonists. In the last reevaluation, PRL level under treatment was within the reference range in 54.7% of the cases, and it was observed complete or partial resolution of the headache in 75% of the cases. The median PRL at this time in patients with complete headache resolution was 17 ng/mL, in those who reported partial recovery was 21 ng/mL, and in those in whom the headache did not change was 66 ng/mL, with a significant difference between the group with complete headache resolution vs. the group with unchanged headache (p=0.022). In the cases with complete headache resolution, the median fall on PRL levels was 89% and in those cases with partial headache resolution 86%, both significantly different (p<0.001) from the fall in the cases with an unchanged headache. CONCLUSION: Data allow us to conclude that, in this series, in the majority of cases the reduction in the level of PRL was followe3d by cessation or relief of the pain.


Assuntos
Cefaleia/sangue , Cefaleia/prevenção & controle , Hiperprolactinemia/terapia , Prolactina/sangue , Adenoma/complicações , Adenoma/terapia , Adulto , Análise de Variância , Agonistas de Dopamina/uso terapêutico , Feminino , Cefaleia/etiologia , Humanos , Hiperprolactinemia/complicações , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/terapia , Valores de Referência , Estatísticas não Paramétricas , Resultado do Tratamento
9.
J Neurooncol ; 146(2): 219-227, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31933258

RESUMO

BACKGROUND: Metastases to the pituitary (MP) are uncommon, accounting for 0.4% of intracranial metastases. Through advances in neuroimaging and oncological therapies, patients with metastatic cancers are living longer and MP may be more frequent. This review aimed to investigate clinical and oncological features, treatment modalities and their effect on survival. METHODS: A systematic review was performed according to PRISMA recommendations. All cases of MP were included, excepted primary pituitary neoplasms and autopsy reports. Descriptive and survival analyses were then conducted. RESULTS: The search identified 2143 records, of which 157 were included. A total of 657 cases of MP were reported, including 334 females (50.8%). The mean ± standard deviation age was 59.1 ± 11.9 years. Lung cancer was the most frequent primary site (31.0%), followed by breast (26.2%) and kidney cancers (8.1%). Median survival from MP diagnosis was 14 months. Overall survival was significantly different between lung, breast and kidney cancers (P < .0001). Survival was impacted by radiotherapy (hazard ratio (HR) 0.49; 95% confidence interval (CI) 0.35-0.67; P < .0001) and chemotherapy (HR 0.58; 95% CI 0.36-0.92; P = .013) but not by surgery. Stereotactic radiotherapy tended to improve survival over conventional radiotherapy (HR 0.66; 95% CI 0.39-1.12; P = .065). Patients from recent studies (≥ 2010) had longer survival than others (HR 1.36; 95% CI 1.05-1.76; P = .0019). CONCLUSION: This systematic review based on 657 cases helped to better identify clinical features, oncological characteristics and the effect of current therapies in patients with MP. Survival patterns were conditioned upon primary cancer histologies, the use of local radiotherapy and systemic chemotherapy, but not by surgery.


Assuntos
Neoplasias/terapia , Neoplasias Hipofisárias/terapia , Padrões de Prática Médica/normas , Terapia Combinada , Humanos , Neoplasias/patologia , Neoplasias Hipofisárias/secundário , Prognóstico
10.
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
11.
J Clin Endocrinol Metab ; 105(3)2020 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-31665485

RESUMO

CONTEXT: The improved remission and complication rates of current transsphenoidal surgery warrant reappraisal of the position of surgery as a viable alternative to dopamine agonists in the treatment algorithm of prolactinomas. OBJECTIVE: To compare clinical outcomes after dopamine agonist withdrawal and transsphenoidal surgery in prolactinoma patients. METHODS: Eight databases were searched up to July 13, 2018. Primary outcome was disease remission after drug withdrawal or surgery. Secondary outcomes were biochemical control and side effects during dopamine agonist treatment and postoperative complications. Fixed- or random-effects meta-analysis was performed to estimate pooled proportions. Robustness of results was assessed by sensitivity analyses. RESULTS: A total of 1469 articles were screened: 55 (10 low risk of bias) on medical treatment (n = 3564 patients) and 25 (12 low risk of bias) on transsphenoidal surgery (n = 1836 patients). Long-term disease remission after dopamine agonist withdrawal was 34% (95% confidence interval [CI], 26-46) and 67% (95% CI, 60-74) after surgery. Subgroup analysis of microprolactinomas showed 36% (95% CI, 21-52) disease remission after dopamine agonist withdrawal, and 83% (95% CI, 76-90) after surgery. Biochemical control was achieved in 81% (95% CI, 75-87) of patients during dopamine agonists with side effects in 26% (95% CI, 13-41). Transsphenoidal surgery resulted in 0% mortality, 2% (95% CI, 0-5) permanent diabetes insipidus, and 3% (95% CI, 2-5) cerebrospinal fluid leakage. Multiple sensitivity analyses yielded similar results. CONCLUSIONS: In the majority of prolactinoma patients, disease remission can be achieved through surgery, with low risks of long-term surgical complications, and disease remission is less often achieved with dopamine agonists.


Assuntos
Procedimentos Clínicos/normas , Agonistas de Dopamina/uso terapêutico , Hipofisectomia/métodos , Microcirurgia/métodos , Neoplasias Hipofisárias/terapia , Prolactinoma/terapia , Agonistas de Dopamina/farmacologia , Agonistas de Dopamina/normas , Feminino , Humanos , Hipofisectomia/efeitos adversos , Hipofisectomia/normas , Microcirurgia/efeitos adversos , Microcirurgia/normas , Hipófise/efeitos dos fármacos , Hipófise/metabolismo , Hipófise/patologia , Hipófise/cirurgia , Neoplasias Hipofisárias/patologia , Guias de Prática Clínica como Assunto , Prolactina/metabolismo , Prolactinoma/patologia , Indução de Remissão/métodos , Estudos Retrospectivos , Resultado do Tratamento
12.
J Clin Endocrinol Metab ; 105(3)2020 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-31701145

RESUMO

PURPOSE: To determine the prevalence of insulin-like growth factor-1 (IGF-1) normalization with long-term multimodality therapy in a pituitary center and to assess changes over time. METHODS: Patients with acromegaly (N = 409), with ≥1 year of data after surgery and at least 2 subsequent clinic visits were included in long-term analysis (N = 266). Biochemical data, clinical characteristics, and therapeutic interventions were reviewed retrospectively. RESULTS: At diagnosis, mean [standard deviation] age was 43.4 [14.3] years, body mass index was 28.5 (24.9-32.1) kg/m2 (median, interquartile range), serum IGF-1 index (IGF-1 level/upper limit of normal) was 2.3 [1.7-3.1], and 80.5% had macroadenomas. Patients with transsphenoidal surgery after 2006 were older [46.6 ± 14.3 vs 40.0 ± 13.4 years; P < 0.001]. Age and tumor size correlated inversely. Overall (N = 266), 93.2% achieved a normal IGF-1 level during 9.9 [5.0-15.0] years with multimodality therapy. The interval to first normal IGF-1 level following failed surgical remission was shorter after 2006: 14.0 (95% confidence interval, 10.0-20.0) versus 27.5 (22.0-36.0) months (P = 0.002). Radiation therapy and second surgery were rarer after 2006: 28 (22%) versus 62 (47.0%); P < 0.001 and 12 (9.4%) versus 28 (21.2%); P = 0.010, respectively. Age at diagnosis increased over time periods, possibly reflecting increased detection of acromegaly in older patients with milder disease. Male gender, older age, smaller tumor and lower IGF-1 index at diagnosis predicted long-term sustained IGF-1 control after surgery without adjuvant therapies. CONCLUSION: The vast majority of patients with acromegaly can be biochemically controlled with multimodality therapy in the current era. Radiotherapy and repeat pituitary surgery became less frequently utilized over time. Long-term postoperative IGF-1 control without use of adjuvant therapies has improved.


Assuntos
Acromegalia/terapia , Adenoma/terapia , Terapia Combinada/métodos , Fator de Crescimento Insulin-Like I/metabolismo , Neoplasias Hipofisárias/terapia , Acromegalia/sangue , Acromegalia/complicações , Adenoma/etiologia , Adulto , Índice de Massa Corporal , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Hipofisárias/etiologia , Período Pós-Operatório , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
13.
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
14.
Horm Metab Res ; 51(12): 755-764, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31826270

RESUMO

With a prevalence of 80-100/100000, pituitary adenomas are more frequent than thought. The rare aggressive pituitary adenoma presents a special challenge, due to the heterogenous presentation of the disease. The prognosis of aggressive pituitary adenomas has been improved due to recent studies demonstrating some efficacy of chemotherapy with temozolomide. However, there is very limited data on second-line therapies in patients with treatment failure. This review presents an update on the diagnostic and therapeutic management of aggressive pituitary tumors. Patients should be treated by a team consisting of an expert endocrinologist, neurosurgeon, radiation oncologist, and pathologist, and according to the recently published ESE guideline.


Assuntos
Neoplasias Hipofisárias/terapia , Animais , Antineoplásicos/administração & dosagem , Humanos , Neoplasias Hipofisárias/tratamento farmacológico , Neoplasias Hipofisárias/radioterapia , Neoplasias Hipofisárias/cirurgia , Temozolomida/administração & dosagem
15.
Childs Nerv Syst ; 35(12): 2273-2278, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31667536

RESUMO

PURPOSE: Craniopharyngiomas remain a challenging entity for neurosurgeons because of their deep-seated, midline location, and intimate relationship with critical neurovascular structures. With high long-term survival rates, patients with craniopharyngioma are likely to experience significant late morbidity related to both disease and therapy. METHOD AND RESULTS: In this paper, we present two cases of late vascular complications after multi-modal treatment of craniopharyngioma. CONCLUSION: Available data suggests that pediatric patients with craniopharyngioma represent a particularly vulnerable group.


Assuntos
Infarto Cerebral/etiologia , Terapia Combinada/efeitos adversos , Craniofaringioma/terapia , Aneurisma Intracraniano/etiologia , Neoplasias Hipofisárias/terapia , Adolescente , Criança , Pré-Escolar , Terapia Combinada/métodos , Feminino , Humanos , Fatores Imunológicos/efeitos adversos , Interferon-alfa/uso terapêutico , Recidiva Local de Neoplasia/terapia , Procedimentos Neurocirúrgicos/efeitos adversos , Procedimentos Neurocirúrgicos/métodos , Complicações Pós-Operatórias/etiologia , Terapia com Prótons/efeitos adversos , Terapia com Prótons/métodos , Radiocirurgia/efeitos adversos , Radiocirurgia/métodos
16.
Curr Urol Rep ; 20(11): 78, 2019 Nov 16.
Artigo em Inglês | MEDLINE | ID: mdl-31734886

RESUMO

PURPOSE OF REVIEW: Hypogonadism is a common endocrine dysfunction. This review focuses on the most up-to-date guideline for evaluation of pituitary function among men presenting with signs and symptoms of hypogonadism. RECENT FINDINGS: The clinician must differentiate between primary (testicular) and secondary (pituitary-hypothalamic or central) hypogonadisms and be aware of adult-onset hypogonadism. If gonadotropins are low or inappropriately normal, the clinician must consider potential reversible causes in the hypothalamus-pituitary axis. Also, it is critical to understand the pitfalls of testosterone testing. When clinically indicated, evaluation of other pituitary hormone functions as well as pituitary magnetic resonance imaging may be necessary. Furthermore, it is essential to recognize that pituitary incidentalomas are common. Patients with microprolactinoma are more likely to present with symptoms of sexual dysfunction while those with macroprolactinoma are more likely to present with symptoms of mass effect. Some functional pituitary tumors respond to drug therapy while other nonfunctional tumors require surgical intervention. It is important for the clinician to understand the proper work-up of the hypogonadal patient with pituitary dysfunction and when necessary to refer to an endocrinologist or a neurosurgeon.


Assuntos
Hipogonadismo/etiologia , Hipófise/fisiopatologia , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/terapia , Prolactinoma/complicações , Testosterona/uso terapêutico , Gonadotropinas/sangue , Humanos , Imagem por Ressonância Magnética , Masculino , Doenças da Hipófise/complicações , Doenças da Hipófise/diagnóstico , Doenças da Hipófise/fisiopatologia , Neoplasias Hipofisárias/diagnóstico , Guias de Prática Clínica como Assunto , Prolactinoma/diagnóstico , Prolactinoma/tratamento farmacológico , Disfunções Sexuais Fisiológicas/etiologia , Doenças Testiculares/sangue , Doenças Testiculares/complicações , Doenças Testiculares/diagnóstico , Testosterona/sangue
17.
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
18.
Neurosurg Clin N Am ; 30(4): 445-455, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31471051

RESUMO

Cavernous sinuses are complex dural venous sinuses that house important neurovascular structures, which often preclude full surgical access for tumor resection. Neuro-imaging and anatomic grading scales have corroborated that more invasive tumors are less likely to undergo gross total resection and biochemical remission. Endoscopic approaches are increasingly favored over microsurgical techniques. Direct transcavernous approaches have yielded even greater degrees of resection. Radiosurgery is a powerful adjuvant therapy for residual, recurrent, and/or inaccessible cavernous sinus disease that provides excellent tumor control rates and favorable risk-benefit ratios for the achievement of biochemical remission with minimal endocrine morbidity.


Assuntos
Adenoma/terapia , Seio Cavernoso/cirurgia , Terapia Combinada/métodos , Neoplasias Hipofisárias/terapia , Humanos , Invasividade Neoplásica , Neuroendoscopia , Radiocirurgia , Resultado do Tratamento
19.
Neurosurg Clin N Am ; 30(4): 473-482, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31471054

RESUMO

Pituitary adenomas are typically slow-growing benign tumors. However, 50% to 60% of tumors progress following subtotal resection and up to 30% recur after apparent complete resection. Options for treatment of recurrent pituitary adenomas include repeat surgical resection, radiation therapy, and systemic therapies. There is no consensus approach for the management of recurrent pituitary adenomas. This article reviews the natural history of recurrent adenomas and emerging biomarkers predictive of clinical behavior as well as the outcomes associated with the various treatment modalities for these challenging tumors, with an emphasis on the surgical treatment.


Assuntos
Adenoma/terapia , Neoplasias Hipofisárias/terapia , Humanos , Recidiva Local de Neoplasia/terapia , Procedimentos Neurocirúrgicos/métodos , Resultado do Tratamento
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