# Primary cutaneous epithelioid hemangioendothelioma of the temporal region: a case report

**Authors:** Xinrong Chen, Yusa Chen, Yuehuan Bian

PMC · DOI: 10.11604/pamj.2025.52.165.50455 · The Pan African Medical Journal · 2025-12-17

## TL;DR

A rare case of skin cancer called epithelioid hemangioendothelioma in the temple area is reported, emphasizing the need for early diagnosis and biopsy.

## Contribution

This case report adds to the limited literature on primary cutaneous EHE, particularly in the head and neck region.

## Key findings

- A 70-year-old woman had a skin lesion on her temple diagnosed as primary cutaneous EHE.
- The diagnosis was confirmed through histopathology and immunohistochemistry showing CD31 and ERG markers.
- The patient showed no disease progression after six months of follow-up without surgery.

## Abstract

Primary cutaneous epithelioid hemangioendothelioma (EHE) is an uncommon low-grade malignant vascular neoplasm that predominantly involves visceral organs, with primary manifestation in the skin, particularly in the head and neck region, being exceptionally rare. This report describes the case of a 70-year-old female presenting with a gradually progressive, intermittently ulcerated plaque on the left temple. Histopathological evaluation revealed characteristic features of EHE, which were further confirmed by strong immunohistochemical expression of endothelial markers CD31 and ERG. Comprehensive systemic assessment demonstrated no evidence of extracutaneous disease. The patient declined surgical intervention due to aesthetic concerns and has been maintained on follow-up every three months. No disease progression has been observed during the six-month follow-up period. This case highlights the importance of including cutaneous epithelioid hemangioendothelioma (EHE) in the differential diagnosis of persistent ulcerative facial lesions and underscores the value of early histopathological biopsy. Early, localized lesions can be completely excised surgically, achieving cure while optimally preserving facial aesthetics.

## Linked entities

- **Proteins:** PECAM1 (platelet and endothelial cell adhesion molecule 1), ERG (ETS transcription factor ERG)
- **Diseases:** epithelioid hemangioendothelioma (MONDO:0015523)

## Full-text entities

- **Genes:** TAFAZZIN (tafazzin, phospholipid-lysophospholipid transacylase) [NCBI Gene 6901] {aka BTHS, CMD3A, EFE, EFE2, G4.5, LVNCX}, BCL2 (BCL2 apoptosis regulator) [NCBI Gene 596] {aka Bcl-2, PPP1R50}, ERG (ETS transcription factor ERG) [NCBI Gene 2078] {aka LMPHM14, erg-3, p55}, CMPK1 (cytidine/uridine monophosphate kinase 1) [NCBI Gene 51727] {aka CK, CMK, CMPK, UMK, UMP-CMPK, UMPK}, CEACAM3 (CEA cell adhesion molecule 3) [NCBI Gene 1084] {aka CD66D, CEA, CGM1, CGM1a, W264, W282}, TFE3 (transcription factor binding to IGHM enhancer 3) [NCBI Gene 7030] {aka MRXSPF, RCCP2, RCCX1, TFEA, bHLHe33}, MAP2K7 (mitogen-activated protein kinase kinase 7) [NCBI Gene 5609] {aka JNKK2, MAPKK7, MEK, MEK 7, MKK7, PRKMK7}, SMN1 (survival of motor neuron 1, telomeric) [NCBI Gene 6606] {aka BCD541, GEMIN1, SMA, SMA1, SMA2, SMA3}, CD68 (CD68 molecule) [NCBI Gene 968] {aka GP110, LAMP4, SCARD1}, CD34 (CD34 molecule) [NCBI Gene 947], PECAM1 (platelet and endothelial cell adhesion molecule 1) [NCBI Gene 5175] {aka CD31, CD31/EndoCAM, GPIIA', PECA1, PECAM-1, endoCAM}, YAP1 (Yes1 associated transcriptional regulator) [NCBI Gene 10413] {aka COB1, YAP, YAP-1, YAP2, YAP65, YKI}, WWTR1 (WW domain containing transcription regulator 1) [NCBI Gene 25937] {aka TAZ}, CAMTA1 (calmodulin binding transcription activator 1) [NCBI Gene 23261] {aka CANPMR, CECBA}, S100A1 (S100 calcium binding protein A1) [NCBI Gene 6271] {aka S100, S100-alpha, S100A}, DES (desmin) [NCBI Gene 1674] {aka CDCD3, CSM1, CSM2, LGMD1D, LGMD1E, LGMD2R}
- **Diseases:** Epithelioid hemangioendothelioma (MESH:D018323), pruritus (MESH:D011537), cutaneous lesion (MESH:D009059), Epithelioid sarcoma (MESH:D012509), ulcerative facial lesions (MESH:D014456), metastasis (MESH:D009362), systemic abnormalities (MESH:D015619), rash (MESH:D005076), vascular neoplasm (MESH:D019043), coagulation (MESH:D001778), borderline malignancy (MESH:D009369), epithelioid hemangioma (MESH:D006391), angiosarcoma (MESH:D006394), lymphadenopathy (MESH:D008206), inflammatory (MESH:D007249), skin lesion (MESH:D012871), bone erosion (MESH:D014077)
- **Chemicals:** thalidomide (MESH:D013792), sirolimus (MESH:D020123), trametinib (MESH:C560077), bevacizumab (MESH:D000068258), sorafenib (MESH:D000077157)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

6 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12995565/full.md

## References

9 references — full list in the complete paper: https://tomesphere.com/paper/PMC12995565/full.md

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Source: https://tomesphere.com/paper/PMC12995565