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Primitive neuroectodermal tumor of the liver: a case report
Siddhartha Mani1, Deep Dutta, Binay K De
1Department of Medicine, Medical College and Hospitals, 88, College Street, Kolkata, West Bengal 700073, India.
Japanese Journal of Clinical Oncology
|December 10, 2009
Summary
This study presents a rare case of primary primitive neuroectodermal tumor of the liver in a young woman. The patient successfully responded to a combination chemotherapy regimen, showing long-term remission.
Area of Science:
- Oncology
- Pathology
Background:
- Primitive neuroectodermal tumors (PNETs) are rare, typically affecting soft tissues.
- Visceral organ involvement by PNETs is uncommon, with liver involvement often being metastatic.
Observation:
- A 20-year-old female presented with massive hepatomegaly.
- Imaging revealed diffuse liver enlargement without biliary involvement.
- Liver biopsy showed small round blue tumor cells expressing Mic-2, Fli-1, and CD-99, with no other primary tumor site identified.
Findings:
- Histopathology and immunohistochemistry confirmed a primary primitive neuroectodermal tumor of the liver.
- The tumor cells were negative for cytokeratin, desmin, OCHIE5, synaptophysin, chromogranin A, and CD-20.
Implications:
- This case highlights the possibility of primary hepatic PNET, a rare diagnosis.
- Combination chemotherapy demonstrated efficacy in treating this rare liver tumor, suggesting a potential treatment strategy.