Spontaneous remission of Cushing's disease: A systematic review.
Ann Endocrinol (Paris)
; 82(6): 613-621, 2021 Dec.
Article
em En
| MEDLINE
| ID: mdl-34687655
ABSTRACT
Spontaneous remission is rare in Cushing's disease. We describe one illustrative case and provide a systematic review of cases previously reported in the literature. Case report A 51-year-old woman diagnosed with Cushing's disease underwent 9 months' isolated metyrapone treatment. Two months after end of treatment, she was admitted with acute kidney failure. After another 4 months, in June 2020, there was no evidence of hypercortisolism, either clinically or biochemically, or of hypocortisolism. At the time of writing, 1 year later, she was still in remission. Cases reported in the literature 23 patients were reported, including the present case. 87% were female with a median age of 32 years. Ten of those with radiologically visible tumors had microadenoma (44%) and 7 had macroadenoma (30%). Mean time from diagnosis to spontaneous remission was 5 months, and was shorter in macroadenoma (1 month) than in microadenoma (13.5 months). Treatments before spontaneous remission were no treatment (65%), steroidogenesis enzyme inhibitors (22%), bilateral adrenalectomy and adrenal autotransplantation (5%), partial bilateral adrenalectomy (4%), and incomplete pituitary surgery (4%). Pituitary tumor apoplexy was the most frequently incriminated event (91%), radiologically documented in 43% of patients. Mean remission during follow-up was 28 months (range, 6-130 months). Recurrence occurred in 39% (n=9) of patients. Although several mechanisms responsible for this phenomenon have been proposed, clinical or subclinical pituitary tumor apoplexy, the latter sometimes presenting atypically, seems to be the most frequently incriminated event. Doctors should be aware of this, and regular follow-up is mandatory due to its unpredictability.
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Base de dados:
MEDLINE
Assunto principal:
Remissão Espontânea
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Hipersecreção Hipofisária de ACTH
Tipo de estudo:
Systematic_reviews
Limite:
Adolescent
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Adult
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Aged
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Ano de publicação:
2021
Tipo de documento:
Article