Outcomes of Therapy for Cushing's Disease due to Adrenocorticotropin-Secreting Pituitary Macroadenomas

Blevins, Lewis S.

doi:10.1210/jc.83.1.63

Cited by 101 publications

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“…In the reported cases CD recurred at least one time in 55% of cases. However, recurrence of benign Cushing's disease after initial surgical cure also occurs in 5-36% of the patients during long-term follow-up [52][53][54][55][56][57]. Furthermore, growth of the pituitary tumor occurred in 67% of the reported cases that were treated with bilateral adrenalectomy as primary treatment.…”

Section: Discussionmentioning

confidence: 99%

Malignant pituitary corticotroph adenomas: report of two cases and a comprehensive review of the literature

et al. 2008

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Corticotroph pituitary carcinomas are tumors, defined by the presence of distant metastases that determine their poor prognosis. The diagnosis and therapy of malignant corticotroph adenomas remains a clinical challenge. The molecular mechanisms of malignant transformation of pituitary adenomas are unclear, although they are believed to arise in an adenoma-to-carcinoma sequence. We describe two cases of malignant Cushing's disease with metastases in liver and bone, respectively. The primary pituitary tumors were treated by a combination of radiotherapy and transsphenoidal surgery, but recurred several times in both patients. The time interval between the diagnosis of Cushing's disease and the discovery of metastases was 32 and 17 years, respectively. In the first case the patient died within 6 months after diagnosis of metastasis, whereas the second patient is alive at a followup of 2 years after the discovery of the metastasis. Furthermore, we reviewed all available cases of corticotroph pituitary carcinomas reported in the literature and analyzed their clinical features and therapeutical management. In conclusion, frequent relapses of Cushing's disease, aggressive growth of macroadenoma, Nelson's syndrome after adrenalectomy or persistently high ACTH levels should prompt the clinician to consider the possibility of pituitary corticotroph carcinomas.

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Section: Discussionmentioning

confidence: 99%

Malignant pituitary corticotroph adenomas: report of two cases and a comprehensive review of the literature

et al. 2008

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“…Initially, we should consider disease characteristics and determine its aggression. Most patients present histological adenoma patterns characterized by sparsely or dense pitu- (1,6,(20)(21)(22)(23)26). Patients who showed a significant gradient between the pituitary (central) and peripheral ACTH values in the inferior petrosal sinus sampling present better prognosis (27).…”

Section: Factors Associated To Cure or Remission Rates In Cushing's Dmentioning

confidence: 99%

“…Although endoscope surgery is less aggressive to bone structures and propose more post-surgical comfort to the patient, there is not enough experience to compare both procedures (29,31). Among experienced neurosurgeons, cure rate in the first surgery in CD is about 83 to 91% in microadenomas and 25 to 73% in macroadenomas (1,2,6,10,22,31,32). Tumor identification in the surgery together with histological adenoma confirmation are associated with better results (1,30,31).…”

Section: Factors Associated To Cure or Remission Rates In Cushing's Dmentioning

confidence: 99%

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Criteria of cure and remission in Cushing's disease: an update

Czepielewski

Rollin

Casagrande

et al. 2007

Arq Bras Endocrinol Metab

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We review the clinical and biochemical criteria used for evaluation of the transsphenoidal pituitary surgery results in the treatment of Cushing's disease (CD). Firstly, we discuss the pathophysiology of the hypothalamic-pituitary-adrenal axis in normal subjects and patients with CD. Considering the series published in the last 25 years, we observed a significant variation in the remission or cure criteria, including the choice of biochemical tests, timing, threshold values to define remission, and the interference of glucocorticoid replacement or previous treatment. In this context we emphasize serum cortisol levels obtained early (from hours to 12 days) in the postoperative period without any glucocorticoid replacement or treatment. Our experience demonstrates that: (i) early cortisol < 5 to 7 µg/dl, (ii) a period of glucocorticoid dependence > 6 mo, (iii) absence of response of cortisol/ACTH to CRH or DDAVP, (iv) return of dexamethasone suppression, and circadian rhythm of cortisol are appropriate indices of remission of CD. In patients with undetectable cortisol levels early after surgery, recurrence seems to be low. Finally, although certain biochemical patterns are more suggestive of remission or surgical failure, none has been proven to be completely accurate, with recurrence observed in approximately 10 to 15% of the patients in long-term follow-up. We recommended that patients with CD should have long-term monitoring of the CRH-ACTH-cortisol axis and associated co-morbidities, especially hypopituitarism, diabetes mellitus, hypertension, cardiovascular disturbances, and osteoporosis. RESUMOCritérios de Cura e Remissão na Doença de Cushing: Uma Atualização. Neste artigo, são revisados os principais critérios clínicos e hormonais utilizados para avaliação do tratamento cirúrgico da Doença de Cushing (DC). Inicialmente são comentados aspectos fisiopatológicos que orientam a avaliação hormonal e os principais fatores clínicos, laboratoriais, cirúrgicos e histológicos associados com melhores resultados, observados nas principais séries da literatura e em pacientes acompanhados prospectivamente pelos autores. Foram revisados, também, critérios adotados nas principais séries da literatura, nos últimos 25 anos, chamando-se atenção para as dosagens hormonais, o momento em que foram realizadas, a possibilidade de interferência de tratamentos prévios e da reposição glicocorticóide. À seguir, essas dosagens são discutidas salientando-se a importância do cortisol obtido seqüencialmente no pós-operatório e sem a interferência de reposição glicocorticóide. A experiência prospectiva dos autores, recentemente referendada na literatura, demonstra que valores de cortisol < 5 a 7 µg/dl associados com um período de dependência aos glicocorticóides > 6 meses, ausência de resposta do ACTH/cortisol ao DDAVP e/ou CRH, retorno da supressão à dexametasona e do ritmo circadiano, estão associados com remissão da DC. Em pacientes com cortisol indetectável após cirurgia transesfenoidal, a chance de recidiva parece ser menor d...

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“…It can affect individuals at any age, but it is most prevalent in the second and third decades of life and is mostly (~80-90%) caused by pituitary tumors with a diameter less than 10 mm (microadenomas) (5)(6)(7).…”

Section: Introductionmentioning

confidence: 99%

Recommendations of the Neuroendocrinology Department of the Brazilian Society of Endocrinology and Metabolism for the diagnosis of Cushing’s disease in Brazil

Machado

Fragoso

Moreira

et al. 2016

Arch. Endocrinol. Metab.

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Although it is a rare condition, the accurate diagnosis and treatment of Cushing's disease is important due to its higher morbidity and mortality compared to the general population, which is attributed to cardiovascular diseases, diabetes mellitus and infections. Screening for hypercortisolism is recommended for patients who present multiple and progressive clinical signs and symptoms, especially those who are considered to be more specific to Cushing's syndrome, abnormal findings relative to age (e.g., spinal osteoporosis and high blood pressure in young patients), weight gain associated with reduced growth rate in the pediatric population and for those with adrenal incidentalomas. Routine screening is not recommended for other groups of patients, such as those with obesity or diabetes mellitus. Magnetic resonance imaging (MRI) of the pituitary, the corticotropin-releasing hormone (CRH) test and the high-dose dexamethasone suppression test are the main tests for the differential diagnosis of ACTH-dependent Cushing's syndrome. Bilateral and simultaneous petrosal sinus sampling is the gold standard method and is performed when the triad of initial tests is inconclusive, doubtful or conflicting. The aim of this article is to provide information on the early detection and establishment of a proper diagnosis of Cushing's disease, recommending follow-up of these patients at experienced referral centers. Arch Endocrinol Metab. 2016;60(3):267-86

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Outcomes of Therapy for Cushing's Disease due to Adrenocorticotropin-Secreting Pituitary Macroadenomas

Cited by 101 publications

References 0 publications

Malignant pituitary corticotroph adenomas: report of two cases and a comprehensive review of the literature

Malignant pituitary corticotroph adenomas: report of two cases and a comprehensive review of the literature

Criteria of cure and remission in Cushing's disease: an update

Recommendations of the Neuroendocrinology Department of the Brazilian Society of Endocrinology and Metabolism for the diagnosis of Cushing’s disease in Brazil

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