Treatment of Nelson's syndrome with temozolomide.

Abstract

A 64-year-old woman was previously treated for Cushing's disease with trans-sphenoidal surgery, external beam radiotherapy and bilateral adrenalectomy. Progression of an aggressive corticotroph adenoma was evident 3 years post-adrenalectomy; involvement of the clivus was treated with surgery and gamma knife radiosurgery. Tumour spread through the skull base, occiput and left ear with persistent facial pain and left ear discharge; progression continued despite second gamma knife treatment. ACTH levels peaked at 2472 and 2265 pmol/l pre- and post-hydrocortisone respectively. Treatment with temozolomide resulted in a significant improvement in symptoms, a reduction of plasma ACTH to 389 pmol/l and regression of tumour on magnetic resonance imaging scan after four cycles of treatment. We propose that temozolomide is an effective and well-tolerated therapeutic tool for the treatment of Nelson's syndrome and a useful addition to the range of therapies available to treat this condition.

• Authors: V J Moyes, G Alusi, H I Sabin, J Evanson, D M Berney, K Kovacs, J P Monson, P N Plowman, W M Drake
• Journal: European journal of endocrinology (Eur J Endocrinol) Vol. 160 Issue 1 Pg. 115-9 (Jan 2009) ISSN: 1479-683X [Electronic] England
• PMID: 18984772 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antineoplastic Agents, Alkylating
• Dacarbazine
• Adrenocorticotropic Hormone
• Temozolomide

Topics

• Adrenocorticotropic Hormone (blood)
• Antineoplastic Agents, Alkylating (therapeutic use)
• Dacarbazine (analogs & derivatives, therapeutic use)
• Female
• Humans
• Middle Aged
• Nelson Syndrome (blood, drug therapy, pathology)
• Temozolomide