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Hepatic perivascular epithelioid cell tumours in children with tuberous sclerosis
  1. Michaela Janks1,
  2. Amelia Heaford2,
  3. Maesha Deheragoda2 and
  4. Nedim Dino Hadzic1
  1. 1Paediatric Liver Medicine Department, King's College Hospital, London, UK
  2. 2Liver Histopathology Laboratory, Institute of Liver Studies, King's College Hospital, London, UK
  1. Correspondence to Dr Michaela Janks; michaela.janks{at}


Perivascular epithelioid cell tumours are a novel histological description of mesenchymal tumours consisting of perivascular and epithelioid cells. Angiomyolipomas are one of the commoner types of this tumour group. They are typically associated with the inherited condition tuberous sclerosis (TS). In TS they are often seen arising in the kidneys and brain, although much more rarely can be seen in the liver. While usually thought of as benign tumours there is little evidence to predict whether they will progress to malignancy. Currently, there is no recommended best management strategy between resection and surveillance. We report two patients with TS seen in our centre with these described PEComa liver lesions histologically, however only one required a resection. On review of the literature, features such as increased size, rapid growth and vascular invasion would be concerning for possible malignant potential and therefore merit resection, as well as significant symptoms.

  • paediatric oncology
  • pathology
  • liver disease

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  • Contributors MJ gathered the case information together and wrote the article. AH added the histopathology information and slides. MD gave advice and guidance on the histopathology sections and NDH gave oversight and guidance to the whole article.

  • 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.

  • Competing interests None declared.

  • Patient consent for publication Parental/guardian consent obtained.

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