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- Title
Malignant perivascular epithelioid cell tumor of the lung synchronous with a primary adenocarcinoma: one case report and review of the literature.
- Authors
Zhao, Jikai; Teng, Haohua; Zhao, Ruiying; Ding, Wenjie; Yu, Keke; Zhu, Lei; Zhang, Jie; Han, Yuchen
- Abstract
<bold>Background: </bold>Perivascular Epithelioid Cell Tumors (PEComa) is an extraordinarily rare mesenchymal neoplasm especially the malignant type originating from the lung. To date, only 8 cases of malignant or malignant potential pulmonary PEComa had been documented. Firm diagnostic criteria for malignant pulmonary PEComa need urgently to be established.<bold>Case Presentation: </bold>We report a challenging case of malignant pulmonary PEComa combined with a primary adenocarcinoma in a 54-year-old man. The PEComa-like tumor showed strong Melan-A and weak transcription factor E3 (TFE3) protein expression but no TFE3 gene rearrangement. The carcinoma-like nodule was recognized as a poorly differentiated primary lung adenocarcinoma.<bold>Discussion and Conclusions: </bold>Our case report was the first case of malignant pulmonary PEComa synchronous with a primary adenocarcinoma and studied the dilemma of diagnosing benign versus malignant criteria for this uncommon tumor.
- Subjects
CELL tumors; LITERATURE reviews; ADENOCARCINOMA; LUNG cancer
- Publication
BMC Cancer, 2019, Vol 19, Issue 1, p1
- ISSN
1471-2407
- Publication type
journal article
- DOI
10.1186/s12885-019-5383-0