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Perivascular epithelioid cell tumor (PEComa) of abdominal cavity from falciform ligament: a case report
Cheol Woong Choi1, Tae Oh Kim, Kyung Yeob Kim
1Department of Internal Medicine, Pusan National University College of Medicine, Busan, Korea.
Abstract:
We present a case of perivascular epithelioid cell tumors (PEComas) in the abdominal cavity at the falciform ligament. A 30-yr-old Korean man visited to hospital for the evaluation of a growing, palpable abdominal mass. He had felt the mass growing over 6 months. There was no family or personal history of tuberous sclerosis. The resected specimen showed a mass of 8.0x7.0x5.5 cm in size. Histological examination showed sheets of spindle-to-epithelioid cells with clear-to-eosinophilic cytoplasm. Immunohistochemically, tumor cells were positive for HMB-4 (gp100) and smooth muscle actin. They were also positive for the S-100, which is a marker of neurogenic and melanocytic tumors. Patient was treated with radical resection of tumor without any adjuvant therapy. He is well and on follow-up visits without tumor recurrence.
Insights
This case study details a rare abdominal perivascular epithelioid cell tumor (PEComa) found in a Korean man. Surgical resection was successful, with no recurrence observed during follow-up.
Area of Science:
- Oncology
- Surgical Pathology
- Rare Tumors
Background:
- Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms.
- These tumors exhibit unique immunophenotypic co-expression of melanocytic and smooth muscle markers.
- PEComas can occur in various anatomic locations, including the abdominal cavity.
Observation:
- A 30-year-old Korean male presented with a growing abdominal mass.
- The mass, located at the falciform ligament, measured 8.0x7.0x5.5 cm.
- Histopathology revealed sheets of spindle-to-epithelioid cells with characteristic cytoplasm.
Findings:
- Immunohistochemistry showed tumor cell positivity for HMB-45 (gp100), smooth muscle actin, and S-100.
- These markers indicate a complex lineage, potentially involving melanocytic and neurogenic differentiation.
- Radical tumor resection was performed without adjuvant therapy.
Implications:
- This case highlights the successful surgical management of a rare abdominal PEComa.
- Complete resection appears to be an effective treatment strategy for localized PEComas.
- Further research into PEComa biology and optimal treatment protocols is warranted.
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