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Hepatic perivascular epithelioid cell tumor: Case report and brief literature review
Da Tang1, Jianmin Wang, Yuepeng Tian
1Department of General Surgery, Second Xiangya Hospital, Central South University Department of General Surgery, the First Hospital of Hunan University of Chinese Medicine, Changsha, Hunan, P.R. China.
Rational:
Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm which expresses both myogenic and melanocytic markers. PEComas are found in a variety locations in the body, but up to now only approximately 30 cases about hepatic perivascular epithelioid cell tumor are reported in English language worldwide.
Patient Concerns:
A 32-year-old woman was admitted in our hospital with intermittent right upper quadrant pain for 1 month and recent (1 day) progressive deterioration.
Diagnoses:
Based on the results of the laboratory examinations and the findings of the computed tomography, the diagnosis of hepatic hamartoma or the hepatocecullar carcinoma with hemorrhage was made.
Interventions:
The patient underwent a segmentectomy of the liver, and the finally diagnosis of hepatic PEComa was made with immunohistochemical confirmation with HMB-45 and SMA.
Outcomes:
There is no clinical or radiographic evidence of recurrence 9 months after surgery.
Lessons:
This kind of tumor is extremely rare and the natural history of PEComa is uncertain, as the treatment protocol for hepatic PEComa has not reached a consensus. But the main treatment of the disease may be surgical resection. Only after long term follow-up can we know whether the tumor is benign or malignant. It appears that longer clinical follow-up is necessary in all patients with hepatic PEComas.
Insights
This case report highlights a rare hepatic perivascular epithelioid cell tumor (PEComa) in a young woman. Surgical resection was successful, with no recurrence at 9 months, emphasizing the need for long-term follow-up.
Area of Science:
- Oncology
- Pathology
- Surgical Oncology
Background:
- Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms with uncertain malignant potential.
- Hepatic PEComas are exceptionally rare, with limited reported cases globally.
- Understanding the characteristics and behavior of hepatic PEComas is crucial due to their rarity.
Observation:
- A 32-year-old woman presented with intermittent right upper quadrant pain and acute deterioration.
- Initial diagnosis considered hepatic hamartoma or hepatocellular carcinoma with hemorrhage based on imaging and labs.
- The patient underwent liver segmentectomy for diagnosis and treatment.
Findings:
- Immunohistochemical analysis confirmed the diagnosis of hepatic PEComa, utilizing markers HMB-45 and SMA.
- Post-operative follow-up at 9 months showed no clinical or radiographic evidence of tumor recurrence.
- The patient's condition stabilized after surgical intervention.
Implications:
- Surgical resection appears to be the primary treatment modality for hepatic PEComa.
- The natural history and definitive treatment protocols for hepatic PEComa remain uncertain.
- Long-term clinical follow-up is essential for all patients diagnosed with hepatic PEComas to monitor for potential recurrence or malignant transformation.
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