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

Abstract

Background: Perivascular epithelioid cell tumor (PEComa) is a mesenchyme tumor derived from soft tissues and organs. Its morphology includes several types such as angiomyolipoma, lymphangiomyomatosis and pulmonary clear-cell “sugar” tumor with similar immunophenotype. Multifocal PEComas are rare lesions, which would confuse imaging examination and clinical management. We reported a case of multifocal angiomyolipomas in the pancreas and liver in a young patient. And we reviewed the literature to explore the clinicopathological characteristics and possible pathogenesis of multifocal PEComas.Case presentation: 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. To explore the nature of the lesion, a liver mass resection and a pancreatic mass biopsy were performed at the first operation. After the postoperative pathology was confirmed as an epithelioid angiomyolipoma, 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. In the diagnosis, in addition to metastatic tumors, multifocal lesions also need to be differentiated with multifocal PEComa; in the treatment, radical treatment should not be performed for this 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.