Filter

Refine by subject

Refine by date

—
  • Print
  • Save
  • Print

Diagnosis and Treatment > Signs and Symptoms

You are looking at 1 - 1 of 1 items for :

  • Virilisation (abnormal) x
Clear All
T O’Shea Department of Endocrinology, Adelaide and Meath Hospitals, incorporating the National Children’s Hospital, Tallaght, Dublin, Ireland

Search for other papers by T O’Shea in
Google Scholar
PubMed Close
,
R K Crowley Department of Endocrinology, Adelaide and Meath Hospitals, incorporating the National Children’s Hospital, Tallaght, Dublin, Ireland

Search for other papers by R K Crowley in
Google Scholar
PubMed Close
,
M Farrell Departments of Neuropathology

Search for other papers by M Farrell in
Google Scholar
PubMed Close
,
S MacNally Departments of Neurosurgery, National Neurosciences Centre, Beaumont Hospital, Beaumont, Dublin, Ireland

Search for other papers by S MacNally in
Google Scholar
PubMed Close
,
P Govender Department of Radiology, Adelaide and Meath Hospitals, incorporating the National Children’s Hospital, Tallaght, Dublin, Ireland

Search for other papers by P Govender in
Google Scholar
PubMed Close
,
J Feeney Department of Radiology, Adelaide and Meath Hospitals, incorporating the National Children’s Hospital, Tallaght, Dublin, Ireland

Search for other papers by J Feeney in
Google Scholar
PubMed Close
,
J Gibney Department of Endocrinology, Adelaide and Meath Hospitals, incorporating the National Children’s Hospital, Tallaght, Dublin, Ireland

Search for other papers by J Gibney in
Google Scholar
PubMed Close
, and
M Sherlock Department of Endocrinology, Adelaide and Meath Hospitals, incorporating the National Children’s Hospital, Tallaght, Dublin, Ireland

Search for other papers by M Sherlock in
Google Scholar
PubMed Close

Summary

Meningioma 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.

Taxonomy:
Clinical Overview, Gland/Organ, Adrenal, Topic, Adrenal, Hormone, 17OHP, Progesterone, Testosterone, Condition/ Syndrome, Congenital adrenal hyperplasia, Hyperandrogenism, Myelolipoma, Neuroendocrine tumour, Sexual development disorders, Patient Demographics, Age, Adult, Gender, Female, Ethnicity, White, Country of Treatment, Ireland, Diagnosis and Treatment, Signs and Symptoms, Abdominal distension, Headache, Proptosis, Virilisation (abnormal), Investigation, 17OHP, ACTH, Androstenedione, CT scan, Histopathology, MRI, Testosterone, Waist circumference, Intervention, Resection of tumour, Medication, Glucocorticoids, Hydrocortisone, Publication Details, Case Report Type, Insight into disease pathogenesis or mechanism of therapy
DOI:
https://doi.org/10.1530/EDM-16-0054
Volume/Issue:
Volume 2016: Issue 1
Open access