# Clinical and pathological analysis of pulmonary epithelioid vascular endothelial tumor

**Authors:** Dong-Yue Wang, Li-Bo Wang, Min Zhang, Jing Wu

PMC · DOI: 10.1515/biol-2025-1259 · Open Life Sciences · 2026-02-12

## TL;DR

This paper discusses the challenges in diagnosing a rare lung tumor called PEHE through two clinical cases and highlights the importance of accurate histopathological and immunohistochemical analysis.

## Contribution

The paper contributes by presenting two diagnostic cases of PEHE and emphasizing the need for improved awareness and diagnostic approaches among clinicians and pathologists.

## Key findings

- PEHE is often misdiagnosed as metastatic or other malignant tumors due to nonspecific clinical and imaging features.
- Accurate diagnosis of PEHE relies on histopathological morphology and immunohistochemical markers like CD34, CD31, and ERG.
- The paper highlights the importance of molecular genetic testing and comprehensive literature review in confirming PEHE diagnosis.

## Abstract

Pulmonary epithelioid hemangioendothelioma (PEHE) is a rare, low-grade malignant vascular tumor with nonspecific clinical and imaging features. Diagnosis remains challenging, particularly in intraoperative frozen sections and biopsy specimens, and relies on characteristic histopathological morphology and immunohistochemical findings, supplemented by molecular genetic testing when necessary. We report two cases of PEHE: a 72-year-old asymptomatic male with multiple pulmonary nodules detected incidentally on CT, initially diagnosed as “malignant tumor, likely metastatic” by frozen section during wedge resection but later confirmed as PEHE on paraffin pathology; and a 53-year-old female presenting with cough, sputum, dyspnea, and chest pain, whose CT showed scattered ground-glass and solid nodules, initially misdiagnosed as non-small cell carcinoma with nodal metastasis via percutaneous biopsy but ultimately confirmed as PEHE through immunohistochemistry (positive CD34/CD31/ERG). These cases underscore the diagnostic pitfalls of PEHE and aim to enhance awareness among clinicians and pathologists. A comprehensive review of current literature is also provided.

## Linked entities

- **Proteins:** CD34 (CD34 molecule), PECAM1 (platelet and endothelial cell adhesion molecule 1), ERG (ETS transcription factor ERG)

## Full-text entities

- **Genes:** TFE3 (transcription factor binding to IGHM enhancer 3) [NCBI Gene 7030] {aka MRXSPF, RCCP2, RCCX1, TFEA, bHLHe33}, CD34 (CD34 molecule) [NCBI Gene 947], VEGFA (vascular endothelial growth factor A) [NCBI Gene 7422] {aka L-VEGF, MVCD1, VEGF, VPF}, ERG (ETS transcription factor ERG) [NCBI Gene 2078] {aka LMPHM14, erg-3, p55}, YAP1 (Yes1 associated transcriptional regulator) [NCBI Gene 10413] {aka COB1, YAP, YAP-1, YAP2, YAP65, YKI}, MDM2 (MDM2 proto-oncogene) [NCBI Gene 4193] {aka ACTFS, HDMX, LSKB, hdm2}, PECAM1 (platelet and endothelial cell adhesion molecule 1) [NCBI Gene 5175] {aka CD31, CD31/EndoCAM, GPIIA', PECA1, PECAM-1, endoCAM}, VIM (vimentin) [NCBI Gene 7431], SMARCB1 (SWI/SNF related BAF chromatin remodeling complex subunit B1) [NCBI Gene 6598] {aka BAF47, CSS3, INI-1, INI1, MRD15, PPP1R144}, CAMTA1 (calmodulin binding transcription activator 1) [NCBI Gene 23261] {aka CANPMR, CECBA}, FLI1 (Fli-1 proto-oncogene, ETS transcription factor) [NCBI Gene 2313] {aka BDPLT21, EWSR2, FLI-1, SIC-1}, CCL2 (C-C motif chemokine ligand 2) [NCBI Gene 6347] {aka GDCF-2, HC11, HSMCR30, MCAF, MCP-1, MCP1}, WWTR1 (WW domain containing transcription regulator 1) [NCBI Gene 25937] {aka TAZ}, TTF1 (transcription termination factor 1) [NCBI Gene 7270] {aka TTF-1, TTF-I}, MUC1 (mucin 1, cell surface associated) [NCBI Gene 4582] {aka ADMCKD, ADMCKD1, ADTKD2, CA 15-3, CD227, Ca15-3}, CMPK1 (cytidine/uridine monophosphate kinase 1) [NCBI Gene 51727] {aka CK, CMK, CMPK, UMK, UMP-CMPK, UMPK}, NKX2-1 (NK2 homeobox 1) [NCBI Gene 7080] {aka BCH, BHC, NK-2, NKX2.1, NKX2A, NMTC1}, PALB2 (partner and localizer of BRCA2) [NCBI Gene 79728] {aka BROVCA5, FANCN, PNCA3}, CDK4 (cyclin dependent kinase 4) [NCBI Gene 1019] {aka CMM3, MCPH31, PSK-J3}, TP63 (tumor protein p63) [NCBI Gene 8626] {aka AIS, B(p51A), B(p51B), EEC3, KET, LMS}, MTOR (mechanistic target of rapamycin kinase) [NCBI Gene 2475] {aka FRAP, FRAP1, FRAP2, RAFT1, RAPT1, SKS}, NAPSA (napsin A aspartic peptidase) [NCBI Gene 9476] {aka KAP, Kdap, NAP1, NAPA, NR1H2-AS1, SNAPA}
- **Diseases:** hemoptysis (MESH:D006469), chest pain (MESH:D002637), lung adenocarcinoma (MESH:D000077192), sclerosing pneumocytoma (MESH:D012598), pleural effusion (MESH:D010996), lymphadenopathy (MESH:D008206), non-small cell carcinoma (MESH:D002289), pulmonary epithelioid angiosarcoma (MESH:D006394), neuroendocrine tumors (MESH:D018358), epithelioid sarcoma (MESH:D012509), pulmonary cryptococcosis (MESH:D003453), metastatic liposarcoma (MESH:D008080), nodules (MESH:D016606), inflammatory (MESH:D007249), anxiety (MESH:D001007), pulmonary epithelioid vascular endothelial tumor (MESH:D019043), adenocarcinoma (MESH:D000230), Epithelioid tumor (MESH:D009369), dyspnea (MESH:D004417), lung cancer (MESH:D008175), pleural invasion (MESH:D010995), metastatic carcinoma (MESH:C538445), Lymph node metastasis (MESH:D008207), metastases (MESH:D009362), anemia (MESH:D000740), EHE (MESH:D018323), weight loss (MESH:D015431), cough (MESH:D003371), P- (MESH:D002972)
- **Chemicals:** bevacizumab (MESH:D000068258), rapamycin (MESH:D020123), paclitaxel (MESH:D017239), carboplatin (MESH:D016190), FDG (MESH:D019788)
- **Species:** Bartonella (genus) [taxon 773], Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

23 references — full list in the complete paper: https://tomesphere.com/paper/PMC12917593/full.md

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Source: https://tomesphere.com/paper/PMC12917593