Poster Presentation ESA-SRB-APEG-NZSE 2022

High dose phenoxybenzamine in a young patient with phaeochromocytoma and likely marijuana withdrawal: A case report (#318)

Jessica Bindra 1 2 , Arunan Sriravindrarajah 1 3 , Albert Hsieh 1 3
  1. Department of Endocrinology and Metabolism, Royal Prince Alfred Hospital, Sydney, NSW, Australia
  2. School of Medicine, Western Sydney University, Sydney, NSW, Australia
  3. Faculty of Medicine and Health, University of Sydney, Sydney, NSW, Australia

Background: Surgical resection of phaeochromocytomas requires careful planning.  The Endocrine Society guidelines recommend combined alpha and beta-adrenergic blockade to reduce peri-operative morbidity and mortality. Phenoxybenzamine is the preferred agent for alpha-adrenergic blockade with a recommended starting dose of 10mg BD, and a maximum suggested dose of 1mg/kg/day (1). There is limited evidence for the use of phenoxybenzamine above this dose.

Case: We present the case of a 23-year-old Caucasian male with a 5cm left-sided phaeochromocytoma and severe hypertension (BP>200/130mmHg), complicated by grade 3 hypertensive retinopathy, macroalbuminuria and left ventricular impairment. He was symptomatic with palpitations, diaphoresis, and headaches. His 24-hour urine normetanephrine was 30.2 umol/24hr (0-3.5) and plasma normetanephrine was 7290 pmol/L (<570). Plasma metanephrine and 3-methoxytyramine were not elevated. Ga-68 DOTATATE-PET/CT confirmed the absence of metastatic disease. The patient engaged in recreational drug use consuming marijuana 1.5g daily, occasional LSD and ketamine.

Despite reaching the recommended 1mg/kg/day of phenoxybenzamine, he continued to have significant hypertension with no postural change. Marijuana withdrawal was considered as a potential contributor to his hypertension, and PRN benzodiazepines were administered. Phenoxybenzamine was progressively uptitrated to a dose of 120mg every 6h with additional Amlodipine 10mg every 12h and Metoprolol 100mg every 12h, to achieve the pre-operative BP 100/70mmHg with a significant postural drop. He was also treated with oral and intravenous sodium chloride prior to surgery. The peri-operative course was uneventful with successful surgical resection, discontinuation of all antihypertensive agents post-operatively and no post-operative hypotension.

Conclusion: This case demonstrates high doses of phenoxybenzamine may be required in young adults with a shorter duration of hypertension and greater arterial compliance, and the challenges of managing marijuana withdrawal in the setting of peri-operative adrenergic blockade for phaeochromocytoma. It highlights the safe administration of high dose phenoxybenzamine in a unique circumstance and the importance of individualised care.

  1. Jacques W. M. Lenders, Quan-Yang Duh, Graeme Eisenhofer, Anne-Paule Gimenez-Roqueplo, Stefan K. G. Grebe, Mohammad Hassan Murad, Mitsuhide Naruse, Karel Pacak, William F. Young, Jr, Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline, The Journal of Clinical Endocrinology & Metabolism, Volume 99, Issue 6, 1 June 2014, Pages 1915–1942, https://doi.org/10.1210/jc.2014-1498