Meningioma in untreated congenital adrenal hyperplasia: a relationship?

Heijboer, Annemieke C.; Netelenbos, J.C.; Blankenstein, Marinus A.

doi:10.1007/s11060-008-9751-x

Cited by 5 publications

(2 citation statements)

References 9 publications

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“…However, there have been increasing case reports of meningiomas seen in association with poorly controlled CAH, postulated to be due to the high levels of sex steroids in these patients. 11,12 The imaging features are similar to sporadic meningiomas and are that of an avidly enhancing extra-axial mass following contrast administration with characteristic osseous hyperostosis ( Figure 5).…”

Section: Gonadsmentioning

confidence: 88%

Imaging features of poorly controlled congenital adrenal hyperplasia in adults

Kok¹,

Sherlock²,

Healy³

et al. 2015

BJR

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Congenital adrenal hyperplasia (CAH) is a genetic autosomal recessive condition most frequently as a result of a mutation in the 21-hydroxylase enzyme gene. Patients with poorly controlled CAH can manifest characteristic imaging findings as a result of adrenocorticotrophic hormone stimulation or the effects of cortisol precursor excess on various target organs. We present a spectrum of imaging findings encountered in adult patients with poorly treated CAH, with an emphasis on radiological features and their clinical relevance.

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Section: Gonadsmentioning

confidence: 88%

Imaging features of poorly controlled congenital adrenal hyperplasia in adults

Kok¹,

Sherlock²,

Healy³

et al. 2015

BJR

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“…To date, there have been two reported cases of growth of PR-expressing meningiomas in patients with congenital adrenal hyperplasia (3, 4). Our patient also has evidence of bilateral giant adrenal myelolipomas, which is likely due to chronic ACTH hyperstimulation.…”

Section: Discussionmentioning

confidence: 99%

Growth of a progesterone receptor-positive meningioma in a female patient with congenital adrenal hyperplasia

O'Shea

Crowley

Farrell³

et al. 2016

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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SummaryMeningioma growth has been previously described in patients receiving oestrogen/progestogen therapy. We describe the clinical, radiological, biochemical and pathologic findings in a 45-year-old woman with congenital adrenal hyperplasia secondary to a defect in the 21-hydroxylase enzyme who had chronic poor adherence to glucocorticoid therapy with consequent virilisation. The patient presented with a frontal headache and marked right-sided proptosis. Laboratory findings demonstrated androgen excess with a testosterone of 18.1 nmol/L (0–1.5 nmol) and 17-Hydroxyprogesterone >180 nmol/L (<6.5 nmol/L). CT abdomen was performed as the patient complained of rapid-onset increasing abdominal girth and revealed bilateral large adrenal myelolipomata. MRI brain revealed a large meningioma involving the right sphenoid wing with anterior displacement of the right eye and associated bony destruction. Surgical debulking of the meningioma was performed and histology demonstrated a meningioma, which stained positive for the progesterone receptor. Growth of meningioma has been described in postmenopausal women receiving hormone replacement therapy, in women receiving contraceptive therapy and in transsexual patients undergoing therapy with high-dose oestrogen and progestogens. Progesterone receptor positivity has been described previously in meningiomas. 17-Hydroxyprogesterone is elevated in CAH and has affinity and biological activity at the progesterone receptor. Therefore, we hypothesise that patients who have long-standing increased adrenal androgen precursor concentrations may be at risk of meningioma growth.Learning points:Patients with long-standing CAH (particularly if not optimally controlled) may present with other complications, which may be related to long-standing elevated androgen or decreased glucocorticoid levels.Chronic poor control of CAH is associated with adrenal myelolipoma and adrenal rest tissue tumours.Meningiomas are sensitive to endocrine stimuli including progesterone, oestrogen and androgens as they express the relevant receptors.

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