Poster Presentation ESA-SRB 2023 in conjunction with ENSA

The Proselyte Pituitary: A tale of a pituitary macroadenoma, or so we thought! (#292)

Daniel Bennett 1 , Kyne Hanlon 1 , Andra Desra 2 , Jennifer Wong 1 3 , Peter Fuller 4 5 6
  1. Endocrinology and Diabetes, Monash Health, Melbourne, VIC, Australia
  2. Clinical Biochemistry, Monash Health, Melbourne, Victoria, Australia
  3. Monash University, Melbourne, Victoria, Australia
  4. Endocrinology, Monash Health, Melbourne, Victoria, Australia
  5. Hudson Institute of Medical Research, Melbourne, VIC, Australia
  6. Monash Health, Melbourne, VIC, Australia

We describe a case of a 48-year-old man who has undergone 4 pituitary surgeries and two courses of radiotherapy for an initially non-functioning but aggressive pituitary adenoma (PA) over a 9-year period. He underwent both a malignant and secretory transformation with pituitary carcinoma (PC) and Cushing’s Disease (CD) after approximately 8 years. He required anti-steroidogenesis therapy with metyrapone as well as bilateral adrenalectomy following sequential admissions to hospital for acute complications of malignant hypertension. He has had a partial response to temozolomide and is planned for Peptide Receptor Radionuclide Therapy (PRRT). His condition is complicated by complete blindness on the right as well as pan-hypopituitarism and primary adrenal insufficiency and he faces significant functional and psychological disability. Monitoring for the development of Nelson’s Syndrome (NS) is of great concern to this man given his significant vision loss.

The rarity of PC and lack of prospective data has made the development of clinical guidelines challenging. In 2018 the European Society of Endocrinology published clinical practice guidelines for the diagnosis and management of aggressive PAs and PCs (1). They include recommendations for pituitary surgery, radiotherapy, endocrine and chemotherapies. Temozolomide is recommended as first line treatment in appropriate cases, with approximately 50% of patients responding. Case series data has shown efficacy for immunotherapy, targeted therapies and PRRT (1, 2).

In 2022 a consensus clinical management guideline was published in Lancet Endocrinology & Diabetes for the diagnosis and management of Cushing's’ disease including advice on medical therapies (3). Metyrapone is suggested for use in situations where rapid lowing of cortisol levels is required to treat acute severe complications Cushing's’ Disease.

Summary

PC is a rare disease which is often functional and can be challenging to treat, requiring a multidisciplinary approach. Options are limited by lack of prospective data but mortality has improved over time.

  1. Raverot G, Burman P, McCormack A, Heaney A, Petersenn S, Popovic V, et al. European Society of Endocrinology Clinical Practice Guidelines for the management of aggressive pituitary tumours and carcinomas. Eur J Endocrinol. 2018;178(1):G1-G24
  2. Du Four S, Van Der Veken J, Duerinck J, Vermeulen E, Andreescu CE, Bruneau M, et al. Pituitary carcinoma - case series and review of the literature. Front Endocrinol (Lausanne). 2022;13:968692.
  3. Fleseriu M, Auchus R, Bancos I, Ben-Shlomo A, Bertherat J, Biermasz NR, et al. Consensus on diagnosis and management of Cushing's disease: a guideline update. Lancet Diabetes Endocrinol. 2021;9(12):847-75
  4. Biglieri EG, Kater CE. Steroid characteristics of mineralocorticoid adrenocortical hypertension. Clin Chem. 1991;37(10 Pt 2):1843-8.