Article Text

PDF
Malignant perivascular epithelioid cell tumour of the fibula: a report and a short review of bone perivascular epithelioid cell tumour
  1. D W Q Lian1,
  2. K L Chuah1,
  3. M H W Cheng2,
  4. W M Yap1
  1. 1Department of Pathology, Tan Tock Seng Hospital, Singapore
  2. 2Department of Orthopaedic Surgery, Tan Tock Seng Hospital, Singapore
  1. Dr K L Chuah, Department of Pathology, Tan Tock Seng Hospital, 11 Jalan Tan Tock Seng, Singapore 308433; gzzckl{at}singnet.com.sg

Abstract

Perivascular epithelioid cell tumour (PEComa) is a term applied to a family of mesenchymal tumours composed of varying proportions of spindle and epithelioid cell components associated with HMB-45 expression. PEComa rarely arises in the soft tissue, visceral organs, skin and bone. This report details an instance when a purely epithelioid PEComa arose from the right fibula of a 52-year-old Chinese woman without features of tuberous sclerosis complex. The excision specimen disclosed an epithelioid tumour with a nested pattern associated with areas of nuclear pleomorphism, mitotic activity, necrosis and vascular invasion in addition to HMB-45 expression on immunohistochemistry. To the best of the authors’ knowledge, this represents the first case of a histologically malignant PEComa of the bone. A short review of primary bone PEComas and potential problems in diagnosis is presented.

Statistics from Altmetric.com

Footnotes

  • Competing interests: None.

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.