SFEBES2013 Poster Presentations Pituitary (71 abstracts)
Growth of a meningioma in a female patient with uncontrolled congenital adrenal hyperplasia
Triona O’Shea 1 , Rachel Crowley 1 , Michael Farrell 2 , Steven Hunter 2 , James Gibney 1 & Mark Sherlock 1
1Tallaght Hospital, Dublin, Ireland; 2Beaumont Hospital, Dublin, Ireland.
Context: Growth of meningiomas has been previously described in patients receiving oestrogen/progestogen therapy.
Methods: Case history, laboratory findings, imaging and histology are discussed.
Case history: A 45-year-old woman with a known history of 21-hydroxyase deficiency (of the non-salt wasting variety) and long-standing non-adherence with corticosteroid therapy presented to the Endocrine Clinic for follow-up care. She complained of severe headache. On examination she was of short stature, virilised and had marked right sided proptosis.
Laboratory findings: Testosterone 19 nmol/l (0–1.5), 17-hydroxyprogesterone >180 nmol/l (<6.5).
Imaging revealed a large left sphenoid wing meningioma with anterior displacement of the right eye.
Histology: Meningioma, Ki index?, MIB-1 index? stained positive for progesterone receptors
Discussion: Growth of meningiomas has been described in patients receiving menopausal hormone therapy, long active reversible contraceptives1 and transsexual patients2. To our knowledge this is the first reported case of aggressive meningioma putatively related to stimulation of the progesterone receptor in response to high levels of 17-hydroxyprogesterone in a patient with uncontrolled congenital adrenal hyperplasia.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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