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  • Acute haemorrhage from a large renal epithelioid angiomyolipoma: diagnostic and management considerations in a teenage patient with a rare cancer

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Case Reports: Rare disease
Acute haemorrhage from a large renal epithelioid angiomyolipoma: diagnostic and management considerations in a teenage patient with a rare cancer
  1. Samantha Conroy1,2,
  2. Jon Griffin3,4,
  3. Marcus Cumberbatch1,2 and
  4. Sanjeev Pathak5
  1. 1Academic Unit of Urology, Department of Oncology and Metabolism, The University of Sheffield, Sheffield, UK
  2. 2Department of Urology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
  3. 3Department of Oncology and Metabolism, The University of Sheffield, Sheffield, UK
  4. 4Department of Pathology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
  5. 5University of Sheffield, Sheffield, UK
  1. Correspondence to Samantha Conroy; s.conroy{at}sheffield.ac.uk

Abstract

Acute renal haemorrhage is a life-threatening condition that is complicated in the context of renal malignancy. Here, we present the case of a teenage male presenting acutely with a large, bleeding renal epithelioid angiomyolipoma (EAML) of the kidney—a rare cancer, which is part of the perivascular epithelioid cell tumour family. The patient was managed acutely with prompt resuscitation, transfer to a centre of expertise and haemorrhagic control using radiologically guided endovascular techniques; this subsequently permitted an oncologically sound procedure (radical nephrectomy, inferior vena cava thrombectomy and lymphadenectomy) to be performed within 24 hours. The description and discussion around this unique case summarises the patient’s clinical journey, while exploring the current literature surrounding diagnosis and outcomes of patients with renal EAMLs.

  • Urological surgery
  • Cancer intervention
  • Urological cancer
  • Genetics
  • Interventional radiology

http://dx.doi.org/10.1136/bcr-2022-252351

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    Footnotes

    • Contributors SC completed all clinical information extraction. JG retrieved and reviewed all histopathological data. All authors (SC, JG, MC and SP) contributed to the writing and editing of the final manuscript submitted. SP was the consultant who directly contributed to the patient’s care and gathered informed consent from the patient involved.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.