Journal
SURGERY TODAY
Volume 44, Issue 3, Pages 572-576Publisher
SPRINGER
DOI: 10.1007/s00595-013-0541-5
Keywords
Retroperitoneal PEComa; Pulmonary lymphangioleiomyomatosis
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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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