title: A rare case of malignant epithelioid angiomyolipoma in multiple locations: multifocal disease or metastases?
abstract: BACKGROUND
  Perivascular epithelioid cell tumors (PEComas), make up a family of extremely rare mesenchymal neoplasms, with characteristic morphological, immunohistochemical and molecular findings. Malignant PEComas and gastrointestinal epithelioid angiomyolipoma (E-AML) are especially rare. To the best of our knowledge E-AML have not been found in the breast. The difficulty in determining what constitutes optimal therapy for PEComas, owing to the sparse literature available, led us to report this rare case.
  
  
  METHODS
  We report a case of a 44-year-old woman, with a family history of multiple endocrine neoplasia syndrome (MEN) (gastrinoma, medullary thyroid cancer and parathyroid hyperplasia), affected by PEComa located in the kidney, stomach, ileum, liver and breast.
  
  
  RESULTS
  The renal, gastric, ileal and mammarian tumors were completely resected, with no evidence of local disease. Liver lesions were biopsied. The morphological and immunohistochemical findings confirm the diagnosis of PEComa.
  
  
  CONCLUSIONS
  On this basis it is difficult to determine if some E-AML are multifocal tumors or metastatic disease.
authors.forename: Daniele
authors.initials: D
authors.lastname: Crocetti
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Pedullà
authors.forename: Maria Rita
authors.initials: MR
authors.lastname: Tarallo
authors.forename: Antonietta
authors.initials: A
authors.lastname: De Gori
authors.forename: Annalisa
authors.initials: A
authors.lastname: Paliotta
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Cavallaro
authors.forename: Giorgio
authors.initials: G
authors.lastname: De Toma
mesh_terms: D018207:Angiomyolipoma / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D001943:Breast Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D018276:Carcinoma, Medullary / Q000235:genetics; D003937:Diagnosis, Differential; D005260:Female; D015408:Gastrinoma / Q000235:genetics; D005770:Gastrointestinal Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D006801:Humans; D006965:Hyperplasia; D007680:Kidney Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D008113:Liver Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D008875:Middle Aged; D018813:Multiple Endocrine Neoplasia Type 2a / Q000235:genetics; D009362:Neoplasm Metastasis / Q000175:diagnosis*; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D010280:Parathyroid Glands / Q000473:pathology; D013964:Thyroid Neoplasms / Q000235:genetics