Poster Presentation ESA-SRB-APEG-NZSE 2022

Staged resection of suspected bilateral phaeochromocytoma in a patient with end-stage kidney disease on haemodialysis (#353)

Brian Tran 1 2 , George Tsihlis 3 , Raymond Lin 2 3 , Nikki L Wong 2 3 , Ravind S Pandher 1 2
  1. Department of Endocrinology, Nepean Hospital, Sydney, New South Wales, Australia
  2. Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia
  3. Department of Renal Medicine, Nepean Hospital, Sydney, New South Wales, Australia

Phaeochromocytoma is a rare finding in end-stage kidney disease (ESKD). Usual treatment complexities are complicated by diagnostic considerations of anuria, dialysis-dependent catecholamine variability and challenges in perioperative fluid management. Plasma metanephrines/normetanephrines can be independently raised in ESKD and haemodialysis, adding further diagnostic challenges. Available case reports of phaeochromocytoma in ESKD have described unilateral lesions. We describe a haemodialysis patient with suspected bilateral phaeochromocytoma, managed with staged surgical resection.

A 73-year-old haemodialysis patient presents with resistant hypertension, on a background of hypertensive nephrosclerosis and Type 2 diabetes mellitus. CT imaging revealed bilateral contrast-enhancing adrenal lesions (left 5.5cm, right 1.4cm) (Figure 1A). PET-CT demonstrated corresponding FDG-avid lesions and PET Gallium DOTATATE was suggestive of bilateral neuroendocrine tumours, although findings were non-specific for the right lesion. (Figure 1B). In the context of resistant hypertension, the lesions were favoured to be phaeochromocytomas, with the diagnosis supported by markedly elevated plasma normetanephrine levels of 6,600pmol/L (Ref <1,079pmol/L).

Blood pressure (BP) control was suboptimal (systolic BP 170-210mmHg) on four anti-hypertensive medications (irbesartan, lercanidipine, atenolol, and prazosin) and were modified to be specific for phaeochromocytoma. Maximum alpha-receptor blockade was achieved with high-dose phenoxybenzamine (300mg/day) followed by propranolol and nifedipine targetting systolic BP 140-160mmHg.

Considering the high perioperative risks and patient comorbidities, a staged unilateral resection was favoured over simultaneous bilateral approach. The left adrenal lesion was resected laparoscopically (Figure 1C) with histopathology confirming phaeochromocytoma (Figure 1D). Minimal vasopressor support for BP was required intraoperatively and no support postoperatively, consistent with bilateral phaeochromocytoma. Interestingly, normetanephrine levels normalised postoperatively despite persistent hypertension, and the decision was made to manage the right lesion with surveillance.

Permanent hypocortisolism occurs following bilateral adrenalectomies. Our case highlights the benefits of a cautious approach to suspected bilateral phaeochromocytomas with staged surgical resection, particularly in high-risk populations and if there is diagnostic imaging uncertainties.

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