ACTH‐independent Cushing's syndrome in pregnancy with spontaneous resolution after delivery: control of the hypercortisolism with metyrapone

Close, Colin; Mann, M.; Watts, J. F.; Taylor, K. G.

doi:10.1111/j.1365-2265.1993.tb02380.x

Cited by 92 publications

(55 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The prevalence of adrenocortical tumors is particularly high during pregnancy, accounting for 70% of all cases of Cushing's syndrome (CS) diagnosed in pregnant women (Guilhaume et al 1992, Lindsay & Nieman 2006, versus only about 25% of cases of CS diagnosed in non-pregnant patients. A few cases of pregnancydependent CS have been reported elsewhere (Caticha et al 1993, Close et al 1993, Wallace et al 1996, Kasperlik-Zaluska et al 2000. We recently observed a worsening of hypercortisolism during pregnancy in a patient with PPNAD, followed by a clear improvement in this condition after spontaneous abortion.…”

Section: Introductionmentioning

confidence: 76%

“…The high frequency of these tumors among hypercortisolic patients during pregnancy (Guilhaume et al 1992) and the description of pregnancy-dependent ACTH-independent CS (Caticha et al 1993, Close et al 1993, Wallace et al 1996, Kasperlik-Zaluska et al 2000 suggest that adrenocortical proliferation and/or secretion may be sensitive to female hormones.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Expression of progesterone and estradiol receptors in normal adrenal cortex, adrenocortical tumors, and primary pigmented nodular adrenocortical disease

Crémoux

Rosenberg²,

Goussard³

et al. 2008

Endocrine Related Cancer

View full text Add to dashboard Cite

Adrenal tumors occur more frequently in women and are the leading cause of Cushing's syndrome during pregnancy. We aimed to evaluate the potential role of sex steroids in the susceptibility of women to adrenocortical tumors. We evaluated the presence of the progesterone receptor (PR), estradiol receptors (ERs), and aromatase in 5 patients with primary pigmented nodular adrenal disease (PPNAD), 15 adrenocortical adenomas (ACAs) and adjacent normal tissues, 12 adrenocortical carcinomas (ACCs), and 3 normal adrenal glands (NA). The expression of PR and ERa was evaluated by enzyme immunoassays, real-time RT-PCR, immunohistochemistry, and cytosol-based ligand-binding assays. ERb and aromatase levels were evaluated by real-time RT-PCR. ERa concentrations were low in NA, in adrenal tissues adjacent to ACA (51G33), in ACC (53G78), and lower in ACA (11G11 fmol/mg DNA). Conversely, PR concentrations were high in NA and adrenal tissues adjacent to ACA, at 307G216 fmol/mg DNA, and were even higher in tumors -726G706 fmol/mg DNA in ACA and 1154G1586 fmol/mg DNA in ACC -and in isolated PPNAD nodules. Binding study results in four tumors were compatible with binding to a steroid receptor. In patients with PPNAD, a strong positive immunohistochemical signal was associated with the sole isolated nodular regions. ERb transcript levels were very high in all samples except those for two ACCs, whereas aromatase levels were low. PR and ERb are clearly present in normal adrenal glands and adrenal tumors. Further studies may shed light on the possible pathogenic role of these receptors in adrenal proliferation.

show abstract

Section: Introductionmentioning

confidence: 76%

Section: Discussionmentioning

confidence: 99%

Expression of progesterone and estradiol receptors in normal adrenal cortex, adrenocortical tumors, and primary pigmented nodular adrenocortical disease

Crémoux

Rosenberg²,

Goussard³

et al. 2008

Endocrine Related Cancer

View full text Add to dashboard Cite

show abstract

“…Confirming this hypothesis, we observed a positive aldosterone response after GNRH and hCG stimulation tests. Cases of ACTH-independent Cushing's syndrome during pregnancy have already been described (11)(12)(13) and an aberrant LHR expression was recently demonstrated in a large adrenal tumor in a pregnant woman with Cushing's syndrome (14).…”

Section: Discussionmentioning

confidence: 99%

A case of primary aldosteronism in pregnancy: do LH and GNRH receptors have a potential role in regulating aldosterone secretion?

Albiger¹,

Sartorato²,

Mariniello³

et al. 2011

European Journal of Endocrinology

View full text Add to dashboard Cite

Objective: The mechanisms inducing steroidogenesis in primary aldosteronism (PA) remain poorly defined. It was recently demonstrated that some G-protein-coupled receptors are abnormally expressed in aldosterone-producing adenomas (APA). We evaluated the potential role of LH and GNRH receptors (LHR (or LHCGR) and GNRHR) in regulating aldosterone secretion in a patient with APA arising during pregnancy (index case) and in a subset of other patients with PA. Patients and methods: GNRH test was performed in the index case, 11 other PA, and 5 controls. GNRHR and LHR expressions were examined in 23 APA and 6 normal tissues. Results: Aldosterone response increased significantly (114%) in the index case after GNRH test was performed preoperatively, while it was blunted after adrenalectomy. Aldosterone also increased after human chorionic gonadotropin and triptorelin stimulation. A partial aldosterone response to GNRH was observed in other 7/11 PA, while a significant response was observed in two patients. Controls did not respond to GNRH test. GNRHR was overexpressed and LHR expression was moderate in the APA tissue from the index case. Moreover, LHR was found in normal adrenals and overexpressed in 6/22 APA. GNRHR was overexpressed in 6/22 APA, 2 of them with a 95-and 109-fold higher expression than normal. A correlation between the clinical and molecular findings was observed in five out of seven patients. Conclusion: We describe a case of PA diagnosed during pregnancy, which appeared to correlate with aberrant LHR and GNRHR expression. Our findings suggest that a subset of patients with PA has aberrant LHR and GNRHR expression, which could modulate aldosterone secretion.

show abstract

“…La cirugía, abierta o laparoscópica (4) debe realizarse en el puerperio (4,5), aunque existen reportes de adrenalectomía unilateral durante el embarazo mediante laparoscopía retroperitoneal (4,12). En espera de la cirugía, o cuando está contraindicada, se ha usado Metyrapone, con buena supresión de cortisol, sin efectos descritos en el feto, en dosis de 2,5 a 3 g día (13)(14)(15). Existen otras alternativas, aunque con probables efectos teratogénicos (16)(17)(18)(19).…”

Section: Figuraunclassified

Síndrome de Cushing durante el embarazo: manifestaciones cutáneas secundarias a un tumor suprarrenal

N¹,

et al. 2013

Rev. chil. obstet. ginecol.

View full text Add to dashboard Cite

RESUMENEl síndrome de Cushing secundario a tumor suprarrenal es una patología infrecuente en el embarazo. Su presencia se traduce en un aumento significativo de la morbimortalidad materna y fetal. Muchas de sus características clínicas son enmascaradas por los cambios propios de una gestación fisiológica, lo que dificulta el diagnóstico. En este contexto las manifestaciones dermatológicas pueden ser de gran utilidad en la sospecha clínica precoz. Se presenta el caso de una paciente de 26 años de edad que en el segundo trimestre de embarazo inicia un cuadro caracterizado por acné inflamatorio severo e hirsutismo. La gestación se interrumpe a las 35 semanas por síndrome hipertensivo del embarazo y rotura prematura de membranas. Durante el puerperio consulta al dermatólogo presentando aún las lesiones descritas, además de estrías violáceas gruesas en abdomen, facie de luna, obesidad centrípeta y máculas purpúricas en zonas de presión. Se diagnóstica síndrome de Cushing. El estudio confirmatorio y etiológico dirigido evidenció un adenoma de corteza suparrenal. El diagnóstico de laboratorio es difícil debido a los cambios físicos y de laboratorio habituales del embarazo que pueden mimetizar los hallazgos propios de la enfermedad. Los hallazgos dermatológicos pueden ser de gran valor para un diagnóstico y tratamiento precoz. Las lesiones dermatológicas secundarias al hipercortisolismo fueron de difícil manejo.PALABRAS CLAVE: Embarazo, hirsutismo, acné, síndrome de Cushing, adenoma adrenal SUMMARY Cushing's syndrome secondary to adrenal tumor is a rare pathology in pregnancy. Its presence results in a significant increase in maternal and fetal morbimortality. Many of its clinical features are masked by the typical changes of a physiological pregnancy, which make difficult the diagnosis. Indeed, skin manifestations may be useful in early clinical suspicion. We report a case of a 26 year old in the second trimester with severe inflammatory acne and hirsutism. The pregnancy is interrupted at 35 weeks for pregnancy-induced hypertension syndrome and premature rupture ovular membranes. During the puerperium, patient consulted a dermatologist presenting still the injuries described, as well as thick purple striae throughout the abdomen, moon face, truncal obesity and purpuric macules in pressure zones. Cushing's syndrome is diagnosed. The study showed a cortex suprarenal adenoma. Laboratory diagnosis is difficult because the pregnancy physical changes and routine laboratory findings that can mimic the disease themselves. Dermatological

show abstract

ACTH‐independent Cushing's syndrome in pregnancy with spontaneous resolution after delivery: control of the hypercortisolism with metyrapone

Cited by 92 publications

References 27 publications

Expression of progesterone and estradiol receptors in normal adrenal cortex, adrenocortical tumors, and primary pigmented nodular adrenocortical disease

Expression of progesterone and estradiol receptors in normal adrenal cortex, adrenocortical tumors, and primary pigmented nodular adrenocortical disease

A case of primary aldosteronism in pregnancy: do LH and GNRH receptors have a potential role in regulating aldosterone secretion?

Síndrome de Cushing durante el embarazo: manifestaciones cutáneas secundarias a un tumor suprarrenal

Contact Info

Product

Resources

About