Perivascular epithelioid cell tumor of the lung: A case report and literature review

Thorac Cancer. 2022 Sep;13(17):2542-2553. doi: 10.1111/1759-7714.14583. Epub 2022 Jul 24.

Abstract

The perivascular epithelioid cell tumor (PEComa) is a rare tumor of interstitial origin characterized by several immunological and muscle cell markers. The clear cell sugar tumor (CCST) of the lung is a type of PEComa defined by thin cell walls and high levels of glycogen in the cytoplasm. We herein reported the case of a 48-year-old male with a recurrence of lung CCST. The preoperative diagnosis of the lung mass was performed by percutaneous needle biopsy. During the thoracoscopic resection, multiple adhesions in the thoracic cavity were described. The tumor invaded the chest wall, and the boundaries between the tumor and surrounding normal tissues were unclear. The mediastinal lymph nodes were significantly enlarged. No relevant gene mutations were detected. After concomitant chemoradiotherapy, the patient's condition was stable. We also conducted a literature review and discussed the overall findings.

Keywords: CCST; PEComa; clear cell sugar tumor; lung; perivascular epithelioid cell tumor.

Publication types

  • Case Reports
  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Biomarkers, Tumor / analysis
  • Biopsy, Needle
  • Humans
  • Lung / pathology
  • Lung Neoplasms* / diagnosis
  • Lung Neoplasms* / genetics
  • Lung Neoplasms* / surgery
  • Lymph Nodes / pathology
  • Male
  • Middle Aged
  • Perivascular Epithelioid Cell Neoplasms* / genetics
  • Perivascular Epithelioid Cell Neoplasms* / surgery

Substances

  • Biomarkers, Tumor