Perivascular epithelioid cell tumor located retroperitoneally with pulmonary lymphangioleiomyomatosis: report of a case

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• 作者：Giacomo Pata (1)
  Andrea Tironi (2)
  Leonardo Solaini (3)
  Travaglia Tiziano (1)
  Fulvio Ragni (1)
  
• 关键词：Retroperitoneal PEComa ; Pulmonary lymphangioleiomyomatosis
• 刊名：Surgery Today
• 出版年：2014
• 出版时间：March 2014
• 年：2014
• 卷：44
• 期：3
• 页码：572-576
• 全文大小：558 KB
• 作者单位：Giacomo Pata (1)
  Andrea Tironi (2)
  Leonardo Solaini (3)
  Travaglia Tiziano (1)
  Fulvio Ragni (1)
  
  1. 2nd Division of General Surgery, Department of Medical and Surgical Sciences, Brescia Civic Hospital, P.le Spedali Civili 1, 25124, Brescia, Italy
  2. Second Department of Pathologic Anatomy, University of Brescia School of Medicine, Brescia, Italy
  3. 2nd Division of General Surgery, Department of Medical and Surgical Sciences, University of Brescia School of Medicine, Brescia, Italy
  
• ISSN：1436-2813

文摘

Perivascular epithelioid cell neoplasms, also known as “PEComas- are unusual mesenchymal tumors, exhibiting perivascular epithelioid cell differentiation and characterized by a mixed myogenic and melanocytic phenotype. “PEComas not otherwise specified-(PEComas-NOS) are especially rare; consequently, there are no published large series, but only case reports. These tumors are rarely located retroperitoneally, with only about 15 such cases reported. We report a case of pulmonary diffuse lymphangioleiomyomatosis with large retroperitoneal PEComa-NOS in a 66-year-old woman. Treatment consisted only of tumor resection, without additional adjuvant therapy. We emphasize the importance of correct immunohistochemistry diagnosis, initiation of recommended treatment, and surveillance of this unique family of tumors.