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Case Reports
. 2022 Dec:101:107781.
doi: 10.1016/j.ijscr.2022.107781. Epub 2022 Nov 17.

Retroperitoneal paraganglioma in a young patient presenting with hypertensive crisis and transient loss of sight; a rare case report and literature review

Kasonde Mulenga  1 , Seke Manase Ephraim Kazuma  2 , James Nonde  3 , Chitani Mbewe  4 , Petrenko Volodymyr  5 , Joseph Musowoya  2
Affiliations
Case Reports

Retroperitoneal paraganglioma in a young patient presenting with hypertensive crisis and transient loss of sight; a rare case report and literature review

Kasonde Mulenga et al. Int J Surg Case Rep. 2022 Dec.

Abstract

Introduction: Catecholamine secreting tumors are a rare type of neuroendocrine tumors whose embryological origin is neural crest cells. 80 % to 90 % arise from the chromaffin cells of adrenal medulla while 10 % to 20 % arise from sympathetic and parasympathetic ganglia. Paragangliomas can be symptomatic due to excess catecholamine secretion or can be asymptomatic. Definitive treatment of paragangliomas is surgical resection.

Case: A 16 yr old male who presented with right iliac fossa pain and hypertension associated with headache and transient loss of sight. Contrasted CT scan of the abdomen revealed a para-aortic retroperitoneal mass, 24 h urine Normetanephrines were significantly elevated. Pre-operative patient preparation included administration of alpha blockers then later beta blockers with adequate hydration. Intra operative blood pressure elevation during tumor manipulation managed with intravenous beta blockers. Post operatively patient required no inotropic support or anti hypertensives. Histopathology revealed features suggestive of a paraganglioma and immunohistochemistry s100 (+) Chromogranin A (+).

Discussion: Functional paragangliomas are manifested by symptoms that result from catecholamine hypersecretion. Biochemical confirmation and functional localization of the tumors is recommended. Definitive management is surgical resection with a pre requisite of adequate patient preparation which includes blood pressure control and volume replacement.

Conclusion: Tumor localisation and adequate patient preparation with alpha blockers and adequate hydration is an important prerequisite to surgical resection.

Keywords: Case report; Hypertension; Paraganglioma; Pheochromocytoma; Retroperitoneal tumor.

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Conflict of interest statement

Declaration of competing interest Authors do not declare any conflicts of interest.

Figures

Image 1
Image 1
A. coronal view CT abdomen (*tumoral mass) (red arrow: Aorta). B. Sagittal view CT abdomen (*tumoral mass) (blue arrow: inferior vena cava). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
Image 2
Image 2
Axial view CT abdomen (*tumoral mass) (blue arrow: inferior vena cava). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
Image 3
Image 3
A. black arrow: tumor before resection. B. Tumor after resection.
Image 4
Image 4
A. After tumor resection (red arrrow: abdominal aorta) (blue arrrow: inferior vena cava). B. After repair of retroperitoneal opening (black arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
Image 5
Image 5
A. Gross pathology specimen. B. Microscopic pathology image H&E stain at magnification ×400.
See this image and copyright information in PMC

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