Spontaneous Remission in Cushing's Disease

Ishibashi, Masayoshi; Shimada, Kenichi; Abe, Kazuhiro; Furue, H; Yamaji, Taketo

doi:10.1001/archinte.1993.00410020097009

Cited by 18 publications

(11 citation statements)

References 21 publications

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“…We observed this phenomenon in only 1.5% of our series of ACTH ad- enoma with Cushing disease. This is consistent with what has been reported in the literature, in which similar cases have been reported very rarely, as described by Dickstein et al 7 and Ishibashi et al 11 It could be argued that some of our cases may be explained by the phenomenon of cyclic cortisol secretion variations (so-called cyclic Cushing syndrome). Cyclic Cushing syndrome has been reported especially in ACTH-independent Cushing syndrome and rarely in ACTH-dependent Cushing syndrome, both in cases of microadenomas and ectopic ACTH secretion.…”

Section: Discussionsupporting

confidence: 93%

ACTH adenomas transforming their clinical expression: report of 5 cases

Zoli¹,

Faustini‐Fustini

Mazzatenta³

et al. 2015

FOC

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OBJECT Adrenocorticotropic hormone (ACTH) adenomas have been recognized as a more aggressive and invasive subtype of pituitary adenomas. An additional and clinically relevant peculiarity of these tumors is their ability to modify their clinical expression from a silent form to Cushing disease or vice versa. The aim of this study was to review a series of patients with pituitary adenomas and analyze the clinical implications of the transformation of clinical expression in 5 cases that showed this phenomenon. METHODS The authors retrospectively reviewed a series of patients with pituitary adenoma and collected clinical, biohumoral, and neuroradiological data of those who presented with a transformation from silent ACTH adenomas to functioning tumors or vice versa. In all the cases, preoperative assessment consisted of brain MRI, ophthalmological examination, and complete baseline endocrinological investigation. In patients with clinical and/or biochemical findings suspicious for Cushing syndrome, a low-dose dexamethasone suppression test was performed to rule in or out this diagnosis. Endocrinological evaluations were repeated 1 month after surgery, 3 months after surgery, and every 6 months or annually thereafter. Ophthalmological evaluations and brain MRIs were repeated after 3 months and then every 6 or 12 months thereafter. RESULTS Five patients (2 men and 3 women) included in this series had corticotropic tumors that showed transformation from an endocrinologically silent form to manifest Cushing disease and vice versa. The mean age at presentation was 40 years (range 18–51 years). In 3 of these patients, a transformation from silent to functioning ACTH adenoma with manifest Cushing disease occurred. In 1 patient, the authors observed the transition from a functioning to a silent adenoma with spontaneous resolution of hypercortisolism. Another patient's silent adenoma “shifted” to a functioning adenoma and then regressed back to a silent form with spontaneous resolution of Cushing disease. This patient again developed hypercortisolism, which finally resolved spontaneously. In this series, the transformation occurred after a mean of 3.5 years (range 6 months to 7 years). The shift from an ACTH-silent to a functioning adenoma was observed in 9% of the ACTH-silent adenomas in this series (4 of 44 cases), and the spontaneous remission of Cushing disease to a silent corticotroph cell adenoma occurred in 1.5% of cases of this series (2 of 132 functioning ACTH adenomas). At follow-up (mean 107 months; range 60–177 months), cortisol levels were within normal limits in all 5 cases. However, 1 patient required Gamma Knife radiosurgery and eventually adrenalectomy for disease control to be achieved. CONCLUSIONS The ability of silent ACTH adenomas to transform their secretion pattern poses a challenge for neurosurgeons and endocrinologists. Because the transformation is often unexpected, the clinical and biochemical data can be underestimated. Furthermore, this bizarre and unpredictable postoperative tumor behavior can lead to misinterpretation of clinical and endocrinological outcomes. Even if these cases are very rare, they are not anecdotal in large series. Thus, ACTH adenomas require careful biohumoral and neuroradiological follow-up to detect possible transformations.

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Section: Discussionsupporting

confidence: 93%

ACTH adenomas transforming their clinical expression: report of 5 cases

Zoli¹,

Faustini‐Fustini

Mazzatenta³

et al. 2015

FOC

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“…Spontaneous remission in Cushing’s syndrome is rare; most cases probably represent infarction of an ACTH-secreting pituitary adenoma (1, 2, 3, 4). Transient resolution of hypercortisolism, with inter-cyclic periods as long as 4 years, is most common in Cushing’s disease (5, 6).…”

Section: Introductionmentioning

confidence: 99%

Prolonged remission after long-term treatment with steroidogenesis inhibitors in Cushing's syndrome caused by ectopic ACTH secretion

Sharma¹,

Nieman²

2012

European Journal of Endocrinology

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Spontaneous remission is rare in ectopic ACTH syndrome (EAS). We describe four patients with presumed EAS in whom long-term treatment with steroidogenesis inhibitors was followed by prolonged remission of hypercortisolemia. Biochemical testing was consistent with EAS, but imaging failed to identify a tumor. Patients were treated with ketoconazole alone or with mitotane and/or metyrapone to control hypercortisolemia. Dexamethasone was added when a block and replace strategy was used. Treatment with steroidogenesis inhibitors for 3–10 years in these patients was followed by a prolonged period of remission (15–60 months). During remission, the first patient had an elevated ACTH, low cortisol and 24-h urinary free cortisol (UFC), and adrenal atrophy on computerized tomography scan during remission, suggesting a direct toxic effect on the adrenal glands. Cases 2 and 3 had normal to low ACTH levels and low-normal UFC, consistent with an effect at the level of the ectopic tumor. They did not have a history of cyclicity and case 3 has been in remission for ~5 years, making cyclic Cushing’s syndrome less likely. Case 4, with a history of cyclic hypercortisolism, had normal to slightly elevated ACTH levels and low-normal UFC during remission. The most likely etiology of remission is cyclic production of ACTH by the ectopic tumor. Spontaneous and sustained remission of hypercortisolemia is possible in EAS after long-term treatment with steroidogenesis inhibitors; a drug holiday may be warranted during chronic therapy to evaluate this. The pathophysiology remains unclear but may involve several different mechanisms.

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“…Finally, several cases of CD that showed initial pituitary apoplexy or spontaneous remission presented recurrence in the follow-up up to seven years after remission (7,8,16,22). Alarifi and cols.…”

Section: Discussionmentioning

confidence: 94%

“…In the subsequent years, some other cases were published and some of them presented recurrence in the follow-up period (7,8,16). However, the cyclic aspect of cortisol secretion that account for 15% of CS patients from several etiologies, mainly CD, are well-described (17).…”

Section: Discussionmentioning

confidence: 99%

Spontaneous remission of hypercortisolism presumed due to asymptomatic tumor apoplexy in ACTH-producing pituitary macroadenoma

Machado

Gadelha

Bronstein

et al. 2013

Arq Bras Endocrinol Metab

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SUMMARYCushing's disease (CD) is usually caused by secretion of ACTH by a pituitary corticotroph microadenoma. Nevertheless, 7%-20% of patients present with ACTH-secreting macroadenomas. Our aim is to report a 36-year-old female patient with CD due to solid-cystic ACTH-macroadenoma followed up during 34 months. The patient presented spontaneous remission due to presumed asymptomatic tumor apoplexy. She showed typical signs and symptoms of Cushing's syndrome (CS). Initial tests were consistent with ACTH-dependent CS: elevated urinary free cortisol, abnormal serum cortisol after low dose dexamethasone suppression test, and elevated midnight salivary cortisol, associated with high plasma ACTH levels. Pituitary magnetic resonance imaging (MRI) showed a sellar mass of 1.2 x 0.8 x 0.8 cm of diameter with supra-sellar extension leading to slight chiasmatic impingement, and showing hyperintensity on T2-weighted imaging, suggesting a cystic component. She had no visual impairment. After two months, while waiting for pituitary surgery, she presented spontaneous resolution of CS. Tests were consistent with remission of hypercortisolism: normal 24-h total urinary cortisol and normal midnight salivary cortisol. Pituitary MRI showed shrinkage of the tumor with disappearance of the chiasmatic compression. She has been free from the disease for 28 months (without hypercortisolism or hypopituitarism). The hormonal and imaging data suggested that silent apoplexy of pituitary tumor led to spontaneous remission of CS. However, recurrence of CS was described in cases following pituitary apoplexy. Therefore, careful long-term follow-up is required. Arq Bras Endocrinol Metab. 2013;57(6):486-9 SUMÁRIO A doença de Cushing (DC) é usualmente causada por um microadenoma produtor de ACTH. Entretanto, 7%-20% dos pacientes apresentam um macroadenoma. O objetivo deste trabalho é reportar uma paciente de 36 anos, feminina, com diagnóstico de DC devido a macroadenoma hipofisário sólido-cístico com seguimento de 34 meses que apresentou remissão espontânea presumidamente em decorrência de uma apoplexia tumoral assintomática. Inicialmente, ela apresentava sinais e sintomas típicos da síndrome de Cushing (SC). Na admissão, os testes foram consistentes com o diagnóstico de SC ACTH-dependente: cortisol urinário livre de 24h elevado, não supressão do cortisol sérico após dose baixa de dexametasona e cortisol salivar noturno elevado, associado a concentrações elevadas do ACTH plasmático. Ressonância magnética (RM) de hipófise revelou uma massa selar de 1.2 x 0.8 x 0.8 cm com extensão suprasselar levando a uma discreta compressão do quiasma óptico e mostrando região de hipersinal na imagem ponderada em T2 sugerindo um componente cístico. A paciente não apresentava queixas visuais. Após dois meses, enquanto aguardava o tratamento cirúrgico, a paciente apresentou remissão espontânea da SC. A repetição dos exames indicou remissão do hipercortisolismo: normalização do cortisol urinário livre de 24h e normalização do cortisol salivar noturno. Nova RM de hipóf...

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Spontaneous Remission in Cushing's Disease

Cited by 18 publications

References 21 publications

ACTH adenomas transforming their clinical expression: report of 5 cases

ACTH adenomas transforming their clinical expression: report of 5 cases

Prolonged remission after long-term treatment with steroidogenesis inhibitors in Cushing's syndrome caused by ectopic ACTH secretion

Spontaneous remission of hypercortisolism presumed due to asymptomatic tumor apoplexy in ACTH-producing pituitary macroadenoma

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