title: Primary perivascular epitheloid cell tumour (PEComa) of the liver: case report and review of the literature.
abstract: OBJECTIVE
  Perivascular epitheloid cell tumour [PEComa] is a rare neoplasm entity, characterized by perivascular epitheloid cells with a coexpression of smooth muscle and melanocytic markers. PEComas are found in a variety of localizations, though lesions within the liver are still scarcely found. Although the majority of these tumours are recognized as benign, there are some reports about advanced and aggressive tumours even with fatal outcome. By means of this case report and literary review including other 21 published cases, potential treatment modalities concerning clinical diagnostics, therapy and the follow-up care should be discussed.
  
  
  METHODS
  The following report presents the case of a 53-year old woman with a known liver lesion, since four years under regularly sonographic controls. Finally, after a haemorrhage episode, the lesion was resected and the diagnosis found. For the literary review a systematic search for case reports published between January 1, 1999 and May 1, 2012 was performed on Pubmed.
  
  
  RESULTS
  The only way, till now, of confirming the diagnosis is through immunohistochemical examinations. The already published Malignancy criteria by Folpe et al. must be taken carefully in question, as there are cases of malignant behaviour, that do not exactly coincide with these.
  
  
  CONCLUSIONS
  Primary PEComa of the liver must be treated as potential malignant and therefore a close follow-up is demanded.
authors.affiliation: Surgery, University of Bonn.
authors.forename: A
authors.initials: A
authors.lastname: Jafari
authors.forename: H P
authors.initials: HP
authors.lastname: Fischer
authors.forename: M
authors.initials: M
authors.lastname: von Websky
authors.forename: G S
authors.initials: GS
authors.lastname: Hong
authors.forename: J C
authors.initials: JC
authors.lastname: Kalff
authors.forename: S
authors.initials: S
authors.lastname: Manekeller
mesh_terms: D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000601:surgery*; D008875:Middle Aged; D054973:Perivascular Epithelioid Cell Neoplasms / Q000145:classification / Q000175:diagnosis* / Q000601:surgery*; D016896:Treatment Outcome