A study of patients with Nelson's syndrome

Pereira, Maria Adelaide Albergaria; Halpern, Alfredo; Salgado, Luiz Roberto; Mendonça, Berenice Bilharinho; Nery, Márcia; Liberman, Bernardo; Streeten, David H. P.; Wajchenberg, B. L.

doi:10.1046/j.1365-2265.1998.00578.x

Cited by 75 publications

(94 citation statements)

References 37 publications

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“…The major concern is the development of Nelson's syndrome in 15-46% of the patients. 5,16,17 Medical therapy in CD mostly serves an adjunctive role after unsuccessful TS. At present only steroidogenesis inhibitors have been proven effective.…”

Section: Introductionmentioning

confidence: 99%

Repeated transsphenoidal pituitary surgery (TS) via the endoscopic technique: a good therapeutic option for recurrent or persistent Cushing's disease (CD)

Wagenmakers¹,

Netea‐Maier²,

Lindert

et al. 2009

Clinical Endocrinology

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SummaryBackground No data on results of repeated transsphenoidal surgery via the endoscopic technique for patients with persistent or recurrent Cushing's disease are available. Design and patients We retrospectively evaluated the remission rates and complications of repeated transsphenoidal surgery via the endoscopic technique in 14 patients with persistent ( N = 6) or recurrent ( N = 8) Cushing's disease treated in our centre between 1999 and 2007. Main outcomes Remission was defined as the disappearance of symptoms of hypercortisolism with basal plasma cortisol level ≤ 50 nmol/l 24-48 h after glucocorticoid withdrawal and/or suppression of plasma cortisol level ≤ 50 nmol/l after 1 mg dexamethasone overnight within the first 3 months after transsphenoidal surgery. Results With repeated endoscopic transsphenoidal surgery a remission rate of 10/14 (71%) was achieved. No patient had a relapse during a median follow-up of 24 months. Cerebrospinal fluid leakage was the most frequent complication (6 patients) and 11 patients required hormonal substitution after surgery. The success of repeated transsphenoidal surgery could not be predicted by visualization of an adenoma on MRI before first or second surgery, histopathological confirmation of an ACTH secreting adenoma after first or second surgery, treatment with cortisol lowering agents before first or second surgery, the operation technique used during the first surgery, persistent vs. recurrent disease after the first surgery, age, gender and interval between the two surgeries. Conclusion Repeated transsphenoidal surgery via the endoscopic technique is a good treatment option for selected patients with recurrent or persistent Cushing's disease following primary pituitary surgery.

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Section: Introductionmentioning

confidence: 99%

Repeated transsphenoidal pituitary surgery (TS) via the endoscopic technique: a good therapeutic option for recurrent or persistent Cushing's disease (CD)

Wagenmakers¹,

Netea‐Maier²,

Lindert

et al. 2009

Clinical Endocrinology

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“…The presence of tumour either at surgery or on imaging has been shown to predict onset, especially if there are aggressive characteristics or macroadenoma (7,12). Young age at the time of adrenalectomy has been suggested to be predictive in some studies (14)(15)(16)(17), but this is not a uniform finding in all (11).…”

Section: Predictive Featuresmentioning

confidence: 78%

“…The duration of Cushing's disease is predictive in some series (18), but again not in all. One area that has attracted considerable interest is whether pituitary radiotherapy either diminishes the chance of developing Nelson's syndrome, or at least delays its onset: it has not been found to be of benefit in several series (6,7,16), whereas other data suggest that prophylactic pituitary radio-therapy at the time of adrenalectomy reduces the risk of progression to Nelson's syndrome (12). Some of the difficulty in establishing the influence of these various factors is the heterogeneity of the series in question, not least of which because of the wide time-frame over which they have been variously reported, and the improvements in recent years in pituitary imaging.…”

Section: Predictive Featuresmentioning

confidence: 99%

Nelson's Syndrome

Munir

Newell‐Price

2007

Arq Bras Endocrinol Metab

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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.

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“…Em 1998 foi publicada a experiência do Serviço de Endocrinologia e Metabologia do HCFMUSP com esta síndrome e, nesta ocasião, 47% de 30 pacientes adrenalectomizados para tratamento de doença de Cushing desenvolveram a síndrome de Nelson (13). Atualmente, temos 33 pacientes adrenalectomizados em acompanhamento há 11,9 ± 6,7 anos e, desses, 17 (52%) desenvolveram a síndrome num tempo médio pós-adrenalectomia de 4,8 ± 2,9 anos (0,9 a 11 anos).…”

Section: Síndrome De Nelsonunclassified

“…Verificamos que a presença de tumor hipofisário no exame de RM magnética é mais freqüente nos pacientes que desenvolveram a síndrome, embora essa diferença não tenha sido significativa do ponto de vista estatístico; apenas dois dos 33 pacientes tinham macroadenoma hipofisário antes da adrenalectomia e ambos desenvolveram síndrome de Nelson após a cirurgia adrenal. Portanto, quanto mais conspícuo for o tumor hipofisário, maior a chance de ocorrer crescimento após a retirada das adrenais (13). Esta constatação também foi feita por outros autores que demonstraram que a identificação do tumor hipofisário, antes da adrenalectomia, é mais freqüente nos pacientes com síndrome de Nelson (15).…”

Section: Síndrome De Nelsonunclassified

Síndrome de Nelson: relato de caso

Cukier

Duch

Teixeira

et al. 2007

Arq Bras Endocrinol Metab

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RESUMOO 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 Apresentação do caso: Dra. Priscilla Cukier (residente de 1º ano do Serviço de Endocrinologia e Metabologia -HCFMUSP)Paciente de 27 anos, do sexo masculino, pardo, com ganho de peso, aparecimento de estrias e acne, arredondamento do rosto, hipertensão arterial e cefaléia occipital matutina há 2 meses. Exame físico: fácies cushingóide, acantose negra cervical, obesidade centrípeta, estrias violáceas em abdome, braços e tronco, acne no dorso, PA= 150 x 100 mmHg e IMC= 33,6kg/m 2 . Avaliação laboratorial: cortisol sérico basal= 38 µg/dl, cortisol sérico 24 horas= 23,1 µg/dl, cortisol sérico após 1 mg dexametasona overnight= 35 µg/dl, cortisol sérico após 8 mg dexametasona overnight= 12 µg/dl, cortisol urinário= 1.217 µg/24 horas e ACTH plasmático= 30,6 pg/ml (normal < 60 pg/ml). Avaliação radiológica: Tomografia computadorizada (TC) crânio: sela balonizada, parcialmente vazia, com imagem sugestiva de microadenoma que deprimia o assoalho e invadia, discretamente, o seio esfenoidal; TC

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A study of patients with Nelson's syndrome

Cited by 75 publications

References 37 publications

Repeated transsphenoidal pituitary surgery (TS) via the endoscopic technique: a good therapeutic option for recurrent or persistent Cushing's disease (CD)

Repeated transsphenoidal pituitary surgery (TS) via the endoscopic technique: a good therapeutic option for recurrent or persistent Cushing's disease (CD)

Nelson's Syndrome

Síndrome de Nelson: relato de caso

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