Multifocal Pancreatic and Liver PEComas Mimic Metastatic Tumors: Case Report and Literature Review

Guan J, Shen G and Huang W

Department of Pathology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, China
Department of Pathology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital & Shenzhen Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, China

^*Correspondance to: Wenting Huang 

 PDF  Full Text Case Report | Open Access

Abstract:

Background: PEComa is a rare type of mesenchymal tumor. Its morphology includes several types such as angiomyolipoma, lymphangiomyomatosis and pulmonary clear-cell “sugar” tumor with similar immunophenotype. Multifocal PEComa is an extremely rare lesion, which can cause confuse both in diagnosis and treatment. In some cases, it may be diagnosed as a metastatic tumor,
and radical treatment is adopted. We reported here a rare case of simultaneous PEComa of the liver and pancreas. At the same time, we reviewed all PEComa cases from 2000 to 2021, found 12 cases of multifocal PEComa, analyzed the clinicopathological characteristics and explored the possible pathogenesis of such lesions.
Case Report: A 28-year-old woman presented with a lesion in the head of the pancreas and liver 5/6 segment respectively, initially suspected of a pancreatic tumor with liver metastasis. A liver mass resection and a pancreatic mass biopsy were performed at the first operation. Both lesions were diagnosed as epithelioid angiomyolipoma. Then the patient underwent a pancreatic segmental
resection. After the operation, the patient did not receive any adjuvant treatment and was followed up for 25 months without tumor recurrence or metastasis.
Conclusion: Multifocal PEComas present a chronic clinical course and have a good prognosis. Surgical resection is the main treatment. The occurrence of sporadic multifocal PEComa may be related to the benign blood dissemination of tumor cells. In the diagnosis, in addition to considering metastatic tumors, multifocal lesions also need to be differentiated from multifocal PEComa; in the treatment, radical treatment should not be performed for such type of disease; in the long-term monitoring of the disease, it is necessary to consider the possibility of a second or a greater number of PEComa lesions even many years later.

Keywords:

Perivascular epithelioid cell tumor (PEComa); Multifocal; Pancreas; Liver

Cite the Article:

Guan J, Shen G, Huang W. Multifocal Pancreatic and Liver PEComas Mimic Metastatic Tumors: Case Report and Literature Review. Clin Oncol. 2023;8:1997..