Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 32
Filtrar
1.
Medicina (Kaunas) ; 58(11)2022 Nov 02.
Artigo em Inglês | MEDLINE | ID: mdl-36363537

RESUMO

Nelson's syndrome is a potentially severe condition that may develop in patients with Cushing's disease treated with bilateral adrenalectomy. Its management can be challenging. Pituitary surgery followed or not by radiotherapy offers the most optimal tumour control, whilst pituitary irradiation alone needs to be considered in cases requiring intervention and are poor surgical candidates. Observation is an option for patients with small lesions, not causing mass effects to vital adjacent structures but close follow-up is required for a timely detection of corticotroph tumour progression and for further treatment if required. To date, no medical therapy has been consistently proven to be effective in Nelson's syndrome. Pharmacotherapy, however, should be considered when other management approaches have failed. A subset of patients with Nelson's syndrome may develop further tumour growth after primary treatment, and, in some cases, a truly aggressive tumour behaviour can be demonstrated. In the absence of evidence-based guidance, the management of these cases is individualized and tailored to previously offered treatments. Temozolomide has been used in patients with aggressive Nelson's with no consistent results. Development of tumour-targeted therapeutic agents are an unmet need for the management of aggressive cases of Nelson's syndrome.


Assuntos
Hormônio Adrenocorticotrópico , Síndrome de Nelson , Humanos , Síndrome de Nelson/diagnóstico , Síndrome de Nelson/etiologia , Síndrome de Nelson/terapia , Adrenalectomia/efeitos adversos , Temozolomida
2.
Endocrinol Metab Clin North Am ; 49(3): 413-432, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32741480

RESUMO

Nelson's syndrome (NS) is a condition which may develop in patients with Cushing's disease after bilateral adrenalectomy. Although there is no formal consensus on what defines NS, corticotroph tumor growth and/or gradually increasing ACTH levels are important diagnostic elements. Pathogenesis is unclear and well-established predictive factors are lacking; high ACTH during the first year after bilateral adrenalectomy is the most consistently reported predictive parameter. Management is individualized and includes surgery, with or without radiotherapy, radiotherapy alone, and observation; medical treatments have shown inconsistent results. A subset of tumors demonstrates aggressive behavior with challenging management, malignant transformation and poor prognosis.


Assuntos
Endocrinologia/tendências , Síndrome de Nelson , Adenoma Hipofisário Secretor de ACT/complicações , Adenoma Hipofisário Secretor de ACT/diagnóstico , Adenoma Hipofisário Secretor de ACT/epidemiologia , Adenoma Hipofisário Secretor de ACT/terapia , Adenoma/complicações , Adenoma/diagnóstico , Adenoma/epidemiologia , Adenoma/terapia , Endocrinologia/métodos , Humanos , Oncologia/métodos , Oncologia/tendências , Síndrome de Nelson/diagnóstico , Síndrome de Nelson/epidemiologia , Síndrome de Nelson/etiologia , Síndrome de Nelson/terapia , Hipersecreção Hipofisária de ACTH/diagnóstico , Hipersecreção Hipofisária de ACTH/epidemiologia , Hipersecreção Hipofisária de ACTH/etiologia , Hipersecreção Hipofisária de ACTH/terapia
3.
J Clin Endocrinol Metab ; 105(5)2020 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-31735971

RESUMO

CONTEXT: Long-term outcomes of patients with Nelson's syndrome (NS) have been poorly explored, especially in the modern era. OBJECTIVE: To elucidate tumor control rates, effectiveness of various treatments, and markers of prognostic relevance in patients with NS. PATIENTS, DESIGN, AND SETTING: Retrospective cohort study of 68 patients from 13 UK pituitary centers with median imaging follow-up of 13 years (range 1-45) since NS diagnosis. RESULTS: Management of Cushing's disease (CD) prior to NS diagnosis included surgery+adrenalectomy (n = 30; eight patients had 2 and one had 3 pituitary operations), surgery+radiotherapy+adrenalectomy (n = 17; two received >1 courses of irradiation, two had ≥2 pituitary surgeries), radiotherapy+adrenalectomy (n = 2), and adrenalectomy (n = 19). Primary management of NS mainly included surgery, radiotherapy, surgery+radiotherapy, and observation; 10-year tumor progression-free survival was 62% (surgery 80%, radiotherapy 52%, surgery+radiotherapy 81%, observation 51%). Sex, age at CD or NS diagnosis, size of adenoma (micro-/macroadenoma) at CD diagnosis, presence of pituitary tumor on imaging prior adrenalectomy, and mode of NS primary management were not predictors of tumor progression. Mode of management of CD before NS diagnosis was a significant factor predicting progression, with the group treated by surgery+radiotherapy+adrenalectomy for their CD showing the highest risk (hazard ratio 4.6; 95% confidence interval, 1.6-13.5). During follow-up, 3% of patients had malignant transformation with spinal metastases and 4% died of aggressively enlarging tumor. CONCLUSIONS: At 10 years follow-up, 38% of the patients diagnosed with NS showed progression of their corticotroph tumor. Complexity of treatments for the CD prior to NS diagnosis, possibly reflecting corticotroph adenoma aggressiveness, predicts long-term tumor prognosis.


Assuntos
Síndrome de Nelson/diagnóstico , Síndrome de Nelson/terapia , Adenoma Hipofisário Secretor de ACT/diagnóstico , Adenoma Hipofisário Secretor de ACT/epidemiologia , Adenoma Hipofisário Secretor de ACT/terapia , Adenoma/diagnóstico , Adenoma/epidemiologia , Adenoma/terapia , Adolescente , Adulto , Idoso , Biomarcadores Tumorais/análise , Criança , Estudos de Coortes , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome de Nelson/epidemiologia , Terapia Neoadjuvante , Prognóstico , Estudos Retrospectivos , Resultado do Tratamento , Reino Unido/epidemiologia , Adulto Jovem
4.
Curr Opin Endocrinol Diabetes Obes ; 22(4): 313-8, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-26087343

RESUMO

PURPOSE OF REVIEW: Nelson's syndrome is a rare complication that can occur during the course of management of Cushing's disease. This article summarizes the recent literature on the diagnosis, monitoring and treatment of this potentially life-threatening outcome. RECENT FINDINGS: Nelson's syndrome, with rising adrenocorticotropin hormone levels and corticotroph tumor progression on diagnostic imaging, can develop following treatment of refractory Cushing's disease with total bilateral adrenalectomy with/without radiotherapy. However, data showing that radiotherapy prevents Nelson's syndrome is inconsistent. In addition to the treatment of Nelson's syndrome with neurosurgery with/without adjuvant radiotherapy, selective somatostatin analogs and dopamine agonists, as well as other novel agents, have been used with increasing frequency in treating cases of Nelson's syndrome with limited benefit. The risk-benefit profile of each of these therapies is still not completely understood. SUMMARY: Consensus guidelines on the evaluation and management of Nelson's syndrome are lacking. This article highlights areas in the surveillance of Cushing's disease patients, and diagnostic criteria and treatment regimens for Nelson's syndrome that require further research and review by experts in the field.


Assuntos
Síndrome de Nelson , Humanos , Síndrome de Nelson/diagnóstico , Síndrome de Nelson/etiologia , Síndrome de Nelson/terapia
5.
Neurosurg Focus ; 38(2): E14, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25639316

RESUMO

Nelson's syndrome is a rare clinical manifestation that occurs in 8%-47% of patients as a complication of bilateral adrenalectomy, a procedure that is used to control hypercortisolism in patients with Cushing's disease. First described in 1958 by Dr. Don Nelson, the disease has since become associated with a clinical triad of hyperpigmentation, excessive adrenocorticotropin secretion, and a corticotroph adenoma. Even so, for the past several years the diagnostic criteria and management of Nelson's syndrome have been inadequately studied. The primary treatment for Nelson's syndrome is transsphenoidal surgery. Other stand-alone therapies, which in many cases have been used as adjuvant treatments with surgery, include radiotherapy, radiosurgery, and pharmacotherapy. Prophylactic radiotherapy at the time of bilateral adrenalectomy can prevent Nelson's syndrome (protective effect). The most promising pharmacological agents are temozolomide, octreotide, and pasireotide, but these agents are often administered after transsphenoidal surgery. In murine models, rosiglitazone has shown some efficacy, but these results have not yet been found in human studies. In this article, the authors review the clinical manifestations, pathophysiology, diagnostic criteria, and efficacy of multimodal treatment strategies for Nelson's syndrome.


Assuntos
Adrenalectomia/efeitos adversos , Síndrome de Nelson/diagnóstico , Síndrome de Nelson/fisiopatologia , Terapia Combinada/métodos , Humanos , Síndrome de Nelson/terapia , Hipersecreção Hipofisária de ACTH/diagnóstico , Hipersecreção Hipofisária de ACTH/cirurgia , Somatostatina/análogos & derivados , Somatostatina/uso terapêutico
6.
World Neurosurg ; 83(6): 1135-40, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25683128

RESUMO

OBJECTIVE: To review the pathophysiology and therapeutic modalities availble for Nelson syndrome. METHODS: We reviewed the current literature including managment for Nelson syndrome. RESULTS: For patients with NS, surgical intervention is often the first-line therapy. With refractory NS or tumors with extrasellar involvement, radiosurgery offers an important alternative or adjuvant option. Pharmacologic interventions have demonstrated limited usefulness, although recent evidence supports the feasibility of a novel somatostatin analog for patients with NS. Modern neuroimaging, improved surgical techniques, and the advent of stereotactic radiotherapy have transformed the management of NS. CONCLUSIONS: An up-to-date understanding of the pathophysiology underlying Nelson Syndrome and evidence-based management is imperative. Early detection may allow for more successful therapy in patients with Nelson Syndrome. Improved radiotherapeutic interventions and rapidly evolving pharmacologic therapies offer an opportunity to create targeted, multifocal treatment regiments for patients with Nelson Syndrome.


Assuntos
Adrenalectomia , Síndrome de Nelson/diagnóstico , Síndrome de Nelson/terapia , Radiocirurgia , Somatostatina/análogos & derivados , Hormônio Adrenocorticotrópico/sangue , Diagnóstico Precoce , Medicina Baseada em Evidências , Humanos , Síndrome de Nelson/metabolismo , Somatostatina/uso terapêutico
7.
Handb Clin Neurol ; 124: 327-37, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25248597

RESUMO

Nelson syndrome is an important complication of treatment with total bilateral adrenalectomy (TBA) for patients with refractory Cushing's disease. Although early cases of Nelson syndrome often presented with the clinical features of large sellar masses, the modern face of Nelson syndrome has changed primarily due to earlier detection (with highly resolved magnetic resonance imaging (MRI) and sensitive ACTH assays) and greater awareness of the condition, resulting in reduced morbidity and mortality. Although lack of administration of neoadjuvant pituitary radiotherapy post-TBA surgery may predict future development of Nelson syndrome, other predictive factors remain controversial. Therefore, Nelson syndrome should be screened for closely and long-term in all patients with a history of Cushing's disease and TBA. The diagnosis of Nelson syndrome remains controversial, and the pathogenesis of this condition is incompletely understood. Current hypotheses include the "released negative feedback" mechansism (residual pituitary corticotropinoma cells are "released" from the negative feedback effects of cortisol following TBA), and the "aggressive corticotropinoma" mechanism (Nelson syndrome is most likely to develop in those patients with refractory treatments - including TBA - for an underlying aggressive corticotropinoma). Effective management of Nelson syndrome with pituitary surgery and radiotherapy is often a challenge. Other therapies (such as Gamma Knife surgery and temozolomide) play an important role and merit further research into their efficacy and placement in the management pathway of Nelson syndrome.


Assuntos
Adrenalectomia/efeitos adversos , Gerenciamento Clínico , Síndrome de Nelson/diagnóstico , Síndrome de Nelson/terapia , Hormônio Adrenocorticotrópico/metabolismo , Animais , Humanos , Síndrome de Nelson/etiologia , Hipersecreção Hipofisária de ACTH/diagnóstico , Hipersecreção Hipofisária de ACTH/cirurgia
8.
J Clin Endocrinol Metab ; 93(7): 2454-62, 2008 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-18413427

RESUMO

OBJECTIVE: Our objective was to evaluate the published literature and reach a consensus on the treatment of patients with ACTH-dependent Cushing's syndrome, because there is no recent consensus on the management of this rare disorder. PARTICIPANTS: Thirty-two leading endocrinologists, clinicians, and neurosurgeons with specific expertise in the management of ACTH-dependent Cushing's syndrome representing nine countries were chosen to address 1) criteria for cure and remission of this disorder, 2) surgical treatment of Cushing's disease, 3) therapeutic options in the event of persistent disease after transsphenoidal surgery, 4) medical therapy of Cushing's disease, and 5) management of ectopic ACTH syndrome, Nelson's syndrome, and special patient populations. EVIDENCE: Participants presented published scientific data, which formed the basis of the recommendations. Opinion shared by a majority of experts was used where strong evidence was lacking. CONSENSUS PROCESS: Participants met for 2 d, during which there were four chaired sessions of presentations, followed by general discussion where a consensus was reached. The consensus statement was prepared by a steering committee and was then reviewed by all authors, with suggestions incorporated if agreed upon by the majority. CONCLUSIONS: ACTH-dependent Cushing's syndrome is a heterogeneous disorder requiring a multidisciplinary and individualized approach to patient management. Generally, the treatment of choice for ACTH-dependent Cushing's syndrome is curative surgery with selective pituitary or ectopic corticotroph tumor resection. Second-line treatments include more radical surgery, radiation therapy (for Cushing's disease), medical therapy, and bilateral adrenalectomy. Because of the significant morbidity of Cushing's syndrome, early diagnosis and prompt therapy are warranted.


Assuntos
Hormônio Adrenocorticotrópico/metabolismo , Síndrome de Cushing/terapia , Síndrome de ACTH Ectópico/terapia , Insuficiência Adrenal/terapia , Adrenalectomia , Humanos , Hipofisectomia , Metirapona/uso terapêutico , Mitotano/uso terapêutico , Síndrome de Nelson/terapia
9.
Arq. bras. endocrinol. metab ; 51(8): 1392-1396, nov. 2007. graf
Artigo em Inglês | LILACS | ID: lil-471756

RESUMO

Nelson's syndrome is a potentially severe complication of bilateral adrenalectomy performed in the treatment of Cushing's disease, and its management remains difficult. Of all of the features of Nelson's syndrome, the one that causes most concern is the development of a locally aggressive pituitary tumour, which, unusually for pituitary disease, may occasionally cause death from the tumour itself. This feature is especially pertinent given the increasing use in Cushing's disease of laparoscopic bilateral adrenal surgery as a highly effective treatment modality to control cortisol-excess. Despite numerous studies and reports, there is no formal consensus of what defines Nelson's syndrome. Thus, some will define Nelson's syndrome according to the classical description with an evolving pituitary mass after bilateral adrenalectomy, whereas others will rely on increasing plasma ACTH levels, even in the absence of a clear pituitary mass lesion on MRI. These factors need to be borne in mind when considering the reports of Nelson's syndrome, as there is great heterogeneity, and it is likely that overall the modern 'Nelson's syndrome' represents a different disease entity from that of the last century. In the present paper, clinical and epidemiological features of Nelson's syndrome, as well as its treatment modalities, are reviewed.


A síndrome de Nelson (SN) é uma complicação potencialmente grave da adrenalectomia bilateral realizada para o tratamento da doença de Cushing e seu manejo permanece difícil. De todas as manifestações da SN, aquela que causa maior preocupação é o desenvolvimento de um tumor hipofisário localmente agressivo, que pode (pouco usualmente para a doença hipofisária) ocasionalmente causar a morte pelo próprio tumor. Este achado é especialmente pertinente, dado o uso cada vez mais freqüente na doença de Cushing, da adrenalectomia bilateral por via laparoscópica, uma modalidade terapêutica altamente efetiva para o controle do excesso de cortisol. Apesar de numerosos estudos e publicações, não existe um consenso formal sobre a definição da SN. Assim, alguns irão definir a SN de acordo com a descrição clássica, como uma massa hipofisária que evolui após a adrenalectomia bilateral, enquanto outros irão se basear nos níveis crescentes dos níveis de ACTH plasmático, mesmo na ausência de uma lesão ou massa hipofisária nitidamente visível à RM. Esses fatores precisam ser relembrados quando se avaliam as publicações sobre SN, na medida em que existe grande heterogeneidade, e é provável que no geral a "SN moderna" represente uma entidade patológica diferente daquela do século passado. Neste artigo, revisaremos achados clínicos e epidemiológicos da SN e também suas modalidades terapêuticas.


Assuntos
Humanos , Síndrome de Nelson , Adrenalectomia/efeitos adversos , Hormônio Adrenocorticotrópico/sangue , Síndrome de Nelson/diagnóstico , Síndrome de Nelson/etiologia , Síndrome de Nelson/terapia , Hipersecreção Hipofisária de ACTH/cirurgia
10.
Neurosurg Focus ; 23(3): E12, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-17961024

RESUMO

The appearance of an adrenocorticotropic hormone (ACTH)-producing tumor after bilateral adrenalectomy for Cushing disease was first described by Nelson in 1958. The syndrome that now bears his name was characterized by hyperpigmentation, a sellar mass, and increased plasma ACTH levels. The treatment of Cushing disease has changed drastically since the 1950s, when the choice was adrenalectomy. Thus, the occurrence, diagnosis, and treatment of Nelson syndrome have changed as well. In the modern era of high-resolution neuroimaging, transsphenoidal microneurosurgery, and stereotactic radiosurgery, Nelson syndrome has become a rare entity. The authors describe the history of the diagnosis and treatment of Nelson syndrome. In light of the changes described, the authors believe this disease must be reevaluated in the contemporary era and a modern paradigm adopted.


Assuntos
Síndrome de Nelson/história , História do Século XX , Humanos , Síndrome de Nelson/diagnóstico , Síndrome de Nelson/terapia
11.
Neurosurg Focus ; 23(3): E5, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-17961026

RESUMO

Cushing's disease is a serious endocrinopathy that, if left untreated, is associated with significant morbidity and mortality rates. After diagnostic confirmation of Cushing's disease has been made, transsphenoidal adenomectomy is the treatment of choice. When a transsphenoidal adenomectomy is performed at experienced transsphenoidal surgery centers, long-term remission rates average 80% overall, surgical morbidity is low, and the mortality rate is typically less than 1%. In patients with well-defined noninvasive microadenomas, the long-term remission rate averages 90%. For patients in whom primary surgery fails, treatment options such as bilateral adrenalectomy, stereotactic radiotherapy or radiosurgery, total hypophysectomy, or adrenolytic medical therapy need to be carefully considered, ideally in a multidisciplinary setting. The management of Nelson's Syndrome often requires both transsphenoidal surgery and radio-therapy to gain disease control.


Assuntos
Adenoma Hipofisário Secretor de ACT/cirurgia , Adenoma/cirurgia , Procedimentos Neurocirúrgicos/métodos , Hipersecreção Hipofisária de ACTH/cirurgia , Seio Esfenoidal/cirurgia , Adenoma Hipofisário Secretor de ACT/patologia , Adenoma/patologia , Humanos , Síndrome de Nelson/terapia , Resultado do Tratamento
12.
Neurosurg Focus ; 23(3): E13, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-17961028

RESUMO

Nelson syndrome (NS) is a rare clinical manifestation of an enlarging pituitary adenoma that can occur following bilateral adrenal gland removal performed for the treatment of Cushing disease. It is characterized by excess adreno-corticotropin secretion and hyperpigmentation of the skin and mucus membranes. The authors present a comprehensive review of the pathophysiology, diagnosis, and management of NS. Corticotroph adenomas in NS remain challenging tumors that can lead to significant rates of morbidity and mortality. A better understanding of the natural history of NS, advances in neurophysiology and neuroimaging, and growing experience with surgical intervention and radiation have expanded the repertoire of treatments. Currently available treatments include surgical, radiation, and medical therapy. Although the primary treatment for each tumor type may vary, it is important to consider all of the available options and select the one that is most appropriate for the individual case, particularly in cases of lesions resistant to intervention.


Assuntos
Síndrome de Nelson , Humanos , Síndrome de Nelson/diagnóstico , Síndrome de Nelson/fisiopatologia , Síndrome de Nelson/terapia , Neurotransmissores/uso terapêutico , Radiocirurgia , Fatores de Risco
13.
Arq Bras Endocrinol Metabol ; 51(1): 116-24, 2007 Feb.
Artigo em Português | MEDLINE | ID: mdl-17435865

RESUMO

The aim of this article is to present and discuss several aspects of the pathogenesis, the clinical, hormonal, and imaging diagnosis, and the treatment of Nelson's syndrome, based on a typical patient's report, in whom several therapeutic approaches were shown to be ineffective.


Assuntos
Adrenalectomia/efeitos adversos , Braquiterapia , Síndrome de Cushing/cirurgia , Síndrome de Nelson/terapia , Adulto , Humanos , Radioisótopos do Iodo/uso terapêutico , Espectroscopia de Ressonância Magnética , Masculino , Síndrome de Nelson/etiologia , Síndrome de Nelson/prevenção & controle
14.
J Clin Endocrinol Metab ; 92(5): 1758-63, 2007 May.
Artigo em Inglês | MEDLINE | ID: mdl-17311852

RESUMO

BACKGROUND: Peroxisome proliferator-activated receptor (PPAR)-gamma agonists have been proposed as therapy to lower plasma ACTH in Cushing's disease. Cyclical secretion of ACTH may, however, explain some of the responses seen. Patients with Nelson's syndrome have persistently high levels of ACTH and may be a better model for examining new therapies to elevated ACTH levels. OBJECTIVE: The objective of the study was to assess whether high-dose rosiglitazone therapy reduces circulating ACTH levels in Nelson's syndrome, a model of ACTH hypersecretion for which no established medical therapy exists. DESIGN: The design was an open-label, prospective, nonrandomized study over 14 wk. SETTING: The study was conducted at a university teaching hospital. PATIENTS: Six patients with Nelson's syndrome participated in the study. METHODS: Patients were assessed at -2, 0, 4, 8, and 12 wk. Rosiglitazone 12 mg/d was administered between 0 and 8 wk. PPAR-gamma immunoreactivity was assessed in pathological tissue. OUTCOME MEASURE: Plasma ACTH was measured before (0830 h) and 120 min after morning dosing with hydrocortisone (HC). RESULTS: One female withdrew prior to commencing therapy for personal reasons. There was no evidence that ACTH levels changed over time (P = 0.864). The average ACTH level was 1187 ng/liter (95% confidence interval 928-1446) for patients before the HC dose and 432 ng/liter (95% confidence interval 172-692) after the HC dose. PPAR-gamma immunoreactivity was positive in three ACTH-secreting tumors available. CONCLUSIONS: Rosiglitazone 12 mg/d did not change circulating ACTH over time, despite PPAR-gamma receptor expression in the tumor tissue. However, this does not preclude the possibility that other patients may respond or that higher doses of rosiglitazone or more potent agonists might prove useful treatment.


Assuntos
Hipoglicemiantes/uso terapêutico , Síndrome de Nelson/tratamento farmacológico , Tiazolidinedionas/uso terapêutico , Adenoma Hipofisário Secretor de ACT/complicações , Adenoma Hipofisário Secretor de ACT/diagnóstico por imagem , Adenoma Hipofisário Secretor de ACT/patologia , Hormônio Adrenocorticotrópico/sangue , Adulto , Idoso , Anti-Inflamatórios/uso terapêutico , Feminino , Fludrocortisona/uso terapêutico , Humanos , Hidrocortisona/uso terapêutico , Imageamento por Ressonância Magnética , Masculino , Síndrome de Nelson/sangue , Síndrome de Nelson/terapia , PPAR gama/biossíntese , PPAR gama/efeitos dos fármacos , Pancreatite/complicações , Hipersecreção Hipofisária de ACTH/complicações , Hipófise/diagnóstico por imagem , Hipófise/patologia , Hipófise/cirurgia , Neoplasias Hipofisárias/complicações , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/patologia , Rosiglitazona , Tomografia Computadorizada por Raios X
15.
Arq. bras. endocrinol. metab ; 51(1): 116-124, fev. 2007. ilus
Artigo em Português | LILACS | ID: lil-448373

RESUMO

O objetivo deste artigo é apresentar e discutir alguns aspectos da patogênese, do diagnóstico clínico, hormonal e radiológico e do tratamento da síndrome de Nelson, com base no relato de um paciente típico portador da doença, no qual várias abordagens terapêuticas mostraram-se ineficazes.


The aim of this article is to present and discuss several aspects of the pathogenesis, the clinical, hormonal, and imaging diagnosis, and the treatment of Nelson's syndrome, based on a typical patient's report, in whom several therapeutic approaches were shown to be ineffective.


Assuntos
Adulto , Humanos , Masculino , Adrenalectomia/efeitos adversos , Braquiterapia , Síndrome de Cushing/cirurgia , Síndrome de Nelson/terapia , Radioisótopos do Iodo/uso terapêutico , Espectroscopia de Ressonância Magnética , Síndrome de Nelson/etiologia , Síndrome de Nelson/prevenção & controle
16.
Arq Bras Endocrinol Metabol ; 51(8): 1392-6, 2007 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-18209878

RESUMO

Nelson's syndrome is a potentially severe complication of bilateral adrenalectomy performed in the treatment of Cushing's disease, and its management remains difficult. Of all of the features of Nelson's syndrome, the one that causes most concern is the development of a locally aggressive pituitary tumour, which, unusually for pituitary disease, may occasionally cause death from the tumour itself. This feature is especially pertinent given the increasing use in Cushing's disease of laparoscopic bilateral adrenal surgery as a highly effective treatment modality to control cortisol-excess. Despite numerous studies and reports, there is no formal consensus of what defines Nelson's syndrome. Thus, some will define Nelson's syndrome according to the classical description with an evolving pituitary mass after bilateral adrenalectomy, whereas others will rely on increasing plasma ACTH levels, even in the absence of a clear pituitary mass lesion on MRI. These factors need to be borne in mind when considering the reports of Nelson's syndrome, as there is great heterogeneity, and it is likely that overall the modern 'Nelson's syndrome' represents a different disease entity from that of the last century. In the present paper, clinical and epidemiological features of Nelson's syndrome, as well as its treatment modalities, are reviewed.


Assuntos
Síndrome de Nelson , Adrenalectomia/efeitos adversos , Hormônio Adrenocorticotrópico/sangue , Humanos , Síndrome de Nelson/diagnóstico , Síndrome de Nelson/etiologia , Síndrome de Nelson/terapia , Hipersecreção Hipofisária de ACTH/cirurgia
18.
Pituitary ; 7(4): 209-15, 2004.
Artigo em Inglês | MEDLINE | ID: mdl-16132203

RESUMO

Adrenalectomy is a radical therapeutic approach to control hypercortisolism in some patients with Cushing's disease. However it may be complicated by the Nelson's syndrome, defined by the association of a pituitary macroadenoma and high ACTH secretion after adrenalectomy. This definition has not changed since the end of the fifties. Today the Nelson's syndrome must be revisited with new to criteria using more sensitive diagnostic tools, especially the pituitary magnetic resonance imaging. In this paper we will review the pathophysiological aspects of corticotroph tumor growth, with reference to the impact of adrenalectomy. The main epidemiological data on the Nelson's syndrome will be presented. More importantly, we will propose a new pathophysiological and practical approach to this question which attempts to evaluate the Corticotroph Tumor Progression after adrenalectomy, rather than to diagnose the Nelson's syndrome. We will discuss the consequences for the management of Cushing's disease patients after adrenalectomy, and will also draw some perspectives.


Assuntos
Adenoma Hipofisário Secretor de ACT/fisiopatologia , Síndrome de Nelson , Neoplasias Hipofisárias/fisiopatologia , Adenoma Hipofisário Secretor de ACT/etiologia , Adenoma Hipofisário Secretor de ACT/terapia , Adrenalectomia/efeitos adversos , Hormônio Adrenocorticotrópico/metabolismo , Adulto , Síndrome de Cushing/fisiopatologia , Síndrome de Cushing/cirurgia , Progressão da Doença , Feminino , Humanos , Síndrome de Nelson/epidemiologia , Síndrome de Nelson/fisiopatologia , Síndrome de Nelson/terapia , Hipersecreção Hipofisária de ACTH/fisiopatologia , Hipersecreção Hipofisária de ACTH/cirurgia , Neoplasias Hipofisárias/etiologia , Neoplasias Hipofisárias/terapia , Prevalência
19.
Am J Clin Oncol ; 14(1): 25-9, 1991 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-1987733

RESUMO

Between 1967 and 1985 95 patients with pituitary adenoma received radiation therapy at the University of California, Los Angeles (UCLA), and the West Los Angeles Veterans' Administration (VA) Medical Center. Seventy of these patients received radiation therapy immediately as adjuvant therapy following incomplete resection, and 25 were irradiated as primary treatment or after surgical failure. The majority of cases (87%) presented clinically with macroadenomas. Two-thirds of the patients were treated with parallel opposed lateral portals to a midline total dose of 50 Gy. The mean follow-up was 7 years (range: 30-250 months). In our series, there is a difference in response by disease subtype. The control rates were 83% (30 of 36) for nonfunctioning adenoma, 60% (nine of 15) for growth-hormone secreting adenoma, 44% (16 of 36) for prolactin secreting adenoma, three of five for Nelson's syndrome, and three of three for Cushing's disease. There was a tendency for a better response rate with a total dose of greater than or equal to 50 Gy for prolactinoma. None of the patients were known to develop brain necrosis or radiation-induced sarcoma. An analysis of these results and a review of the literature is presented here.


Assuntos
Adenoma/radioterapia , Neoplasias Hipofisárias/radioterapia , Acromegalia/terapia , Adenoma/patologia , Adenoma/cirurgia , Adenoma Cromófobo/terapia , Adolescente , Adulto , Idoso , Criança , Terapia Combinada , Síndrome de Cushing/terapia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome de Nelson/terapia , Estadiamento de Neoplasias , Neoplasias Hipofisárias/patologia , Neoplasias Hipofisárias/cirurgia , Prolactinoma/terapia , Dosagem Radioterapêutica , Estudos Retrospectivos
20.
Obstet Gynecol ; 71(6 Pt 2): 1053-6, 1988 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-3374920

RESUMO

In a patient with Nelson's syndrome, elevated peripheral concentrations of androgens usually associated with neoplasm prompted ovarian vein catheterization. Androgen excess was limited to the right ovary. However, laparotomy revealed bilateral multiple paraovarian nodules with histologic appearance of adrenocortical tissue. The occurrence of virilization from ectopic adrenal tissue with markedly elevated ACTH concentrations is exceedingly rare.


Assuntos
Síndrome de Nelson/complicações , Neoplasias Ovarianas/etiologia , Neoplasias Hipofisárias/complicações , Virilismo/etiologia , Adulto , Androgênios/metabolismo , Terapia Combinada , Feminino , Humanos , Síndrome de Nelson/terapia , Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/cirurgia
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...