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Peer-reviewedCC BY 4.0ACG Case Reports Journal

Hepatic Epithelioid Angiosarcoma with Abberrant Synaptophysin and CDX2 Expression Mimicking Neuroendocrine/Metastatic Carcinoma: A Fatal Case Report

5 affiliations
  1. 1Mercy Hospital, Fort Smith, AR
  2. 2Arkansas College of Osteopathic Medicine, Fort Smith, AR
  3. 3Liaquat University of Medical and Health Sciences, Jamshoro, Pakistan
  4. 4King Edward Medical University, Lahore, Pakistan
  5. 5Allama Iqbal Medical College, Lahore, Pakistan

Abstract

We report a fatal case of presumed primary hepatic epithelioid angiosarcoma (PHEA) in an 82-year-old woman with back pain, abdominal distension, and hyponatremia. Imaging showed ascites and hypodense hepatic lesions. Ascitic cytology was negative. Liver biopsy showed malignant cells with positive erythroblast transformation-specific regulated gene 1 (ERG) and cluster of differentiation 31 (CD31) on immunohistochemistry. Patchy synaptophysin and caudal-type homeobox 2 positivity initially suggested a neuroendocrine tumor but proved aberrant; negative chromogranin with ERG/CD31 positivity supported PHEA. She was transitioned to hospice care due to rapid clinical decline. This case highlights the atypical presentation, diagnostic pitfalls, and aggressive clinical course of PHEA.

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BibTeX
@article{usman2026hepatic,
  title = {{Hepatic Epithelioid Angiosarcoma with Abberrant Synaptophysin and CDX2 Expression Mimicking Neuroendocrine/Metastatic Carcinoma: A Fatal Case Report}},
  author = {Muhammad Usman and Muhammad Usman Shahbaz and Arsalan Ahmed and Muahammad Zawar Asif and Adnan Memon and Laiba Murtaza and Rafif Ahmed},
  journal = {ACG Case Reports Journal},
  year = {2026},
  month = nov,
  doi = {10.14309/crj.0000000000002389},
  url = {https://doi.org/10.14309/crj.0000000000002389},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Hepatic Epithelioid Angiosarcoma with Abberrant Synaptophysin and CDX2 Expression Mimicking Neuroendocrine/Metastatic Carcinoma: A Fatal Case Report
AU  - Muhammad Usman
AU  - Muhammad Usman Shahbaz
AU  - Arsalan Ahmed
AU  - Muahammad Zawar Asif
AU  - Adnan Memon
AU  - Laiba Murtaza
AU  - Rafif Ahmed
PY  - 2026
DA  - 2026/11/04
JO  - ACG Case Reports Journal
DO  - 10.14309/crj.0000000000002389
UR  - https://doi.org/10.14309/crj.0000000000002389
AB  - We report a fatal case of presumed primary hepatic epithelioid angiosarcoma (PHEA) in an 82-year-old woman with back pain, abdominal distension, and hyponatremia. Imaging showed ascites and hypodense hepatic lesions. Ascitic cytology was negative. Liver biopsy showed malignant cells with positive erythroblast transformation-specific regulated gene 1 (ERG) and cluster of differentiation 31 (CD31) on immunohistochemistry. Patchy synaptophysin and caudal-type homeobox 2 positivity initially suggested a neuroendocrine tumor but proved aberrant; negative chromogranin with ERG/CD31 positivity supported PHEA. She was transitioned to hospice care due to rapid clinical decline. This case highlights the atypical presentation, diagnostic pitfalls, and aggressive clinical course of PHEA.
SN  - 2326-3253
ER  -