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

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Abstract

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.

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Fig. 1
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Abbreviations

PEComas-NOS:

PEComas-not otherwise specified

LAM:

Lymphangioleiomyomatosis

HRCT:

High-resolution computed tomography

HPF:

High power fields

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Conflict of interest

No author has any conflict of interest to declare.

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Authors and Affiliations

  1. 2nd Division of General Surgery, Department of Medical and Surgical Sciences, Brescia Civic Hospital, P.le Spedali Civili 1, 25124, Brescia, Italy

    Giacomo Pata, Travaglia Tiziano & Fulvio Ragni

  2. Second Department of Pathologic Anatomy, University of Brescia School of Medicine, Brescia, Italy

    Andrea Tironi

  3. 2nd Division of General Surgery, Department of Medical and Surgical Sciences, University of Brescia School of Medicine, Brescia, Italy

    Leonardo Solaini

Authors
  1. Giacomo Pata
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  2. Andrea Tironi
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  3. Leonardo Solaini
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  4. Travaglia Tiziano
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  5. Fulvio Ragni
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Correspondence to Giacomo Pata.

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Pata, G., Tironi, A., Solaini, L. et al. Perivascular epithelioid cell tumor located retroperitoneally with pulmonary lymphangioleiomyomatosis: report of a case. Surg Today 44, 572–576 (2014). https://doi.org/10.1007/s00595-013-0541-5

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  • DOI: https://doi.org/10.1007/s00595-013-0541-5

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