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Case Reports
. 2016 May 10:2016:bcr2016214719.
doi: 10.1136/bcr-2016-214719.

Unusual presentation of phaeochromocytoma

D C D Hope  1 , J M Palan  1
Affiliations
Case Reports

Unusual presentation of phaeochromocytoma

D C D Hope et al. BMJ Case Rep. .

Abstract

A 44-year-old woman, with a background of heart, lung and renal transplantation secondary to cystic fibrosis and type 1 diabetes, presented with tachycardia, hyperglycaemia, nausea and vomiting. She was initially managed for diabetic ketoacidosis with severe dehydration. However, persistent episodic hypertension and tachycardia led the investigating team to identify significantly raised urinary metanephrines and a left-sided adrenal mass; Iodine-123-meta-iodobenzylguanidine single photon emission computer tomography scan (MIBG SPECT/CT) showed avid uptake of tracer, confirming a left-sided phaeochromocytoma. She was started on medical management and is awaiting an elective laparoscopic procedure. This case describes the presentation of a unilateral phaeochromocytoma as ketoacidosis in a patient with type 1 diabetes with no other apparent precipitant. This highlights the metabolic counter-regulatory effect of excess catecholamines in addition to the inotropic/chronotropic effects that are associated with this adrenal tumour. Recognition of atypical signs and symptoms may point towards an atypical precipitant of diabetic ketoacidosis-a medical emergency presenting to front-line clinicians.

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Figures

Figure 1
Figure 1
CT scan showing left-sided partially necrotic adrenal mass (axial plane).
Figure 2
Figure 2
CT scan showing left-sided partially necrotic adrenal mass (coronal plane).
Figure 3
Figure 3
123I-MIBG SPECT/CT scan showing avid uptake of tracer in the left adrenal gland. (A) Axial plane (B) sagittal plane and (C) coronal plane.
See this image and copyright information in PMC

References

    1. Lenders JW, Eisenhofer G, Mannelli M et al. . Phaeochromocytoma. Lancet 2005;366:665–75. 10.1016/S0140-6736(05)67139-5 - DOI - PubMed
    1. Castinetti F, Taieb D, Henry JF et al. . Management of endocrine disease: outcome of adrenal sparing surgery in heritable pheochromocytoma. Eur J Endocrinol 2016;174:R9–R18. 10.1530/EJE-15-0549 - DOI - PubMed
    1. Stein PP, Black HR. A simplified diagnostic approach to pheochromocytoma. A review of the literature and report of one institutions experience. Medicine 1991;70:46–66. - PubMed
    1. Eisenhofer G, Lenders JW, Siegert G et al. . Plasma methoxytyramine: a novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status. Eur J Cancer 2012;48:1739–49. - PMC - PubMed
    1. Douma S, Petidis K, Kartali N et al. . Pheochromocytoma presenting as diabetic ketoacidosis. J Diabetes Complications 2008;22:295–6. 10.1016/j.jdiacomp.2007.02.006 - DOI - PubMed

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