Perivascular Epithelioid Cell Tumor: The First Malignant Case Report in the Pancreas
摘要：
Pancreatic perivascular epithelioid cell tumors (PEComas) are exceedingly rare neoplasms, < 10 cases have been described; all were benign and occurring mostly in women. We report the first malignant pancreatic PEComa in a patient with BRCA2 mutation. A 51-year-old woman with a medical and familial history of breast adenocarcinoma, who was tested positive for BRCA2 mutation, presented with a recent history of jaundice. Imaging studies were consistent with endocrine or secondary tumor. Pathologic examination of fine needle aspiration biopsy and Whipple procedure revealed a 6-cm intrapancreatic PEComa, invading the duodenal wall with extensive necrosis and vascular invasion, leading to classify this PEComa into "malignant" category. The patient developed liver metastases 6 months after surgery. Although mostly benign, thorough sampling of the PEComa is mandatory, to rule out malignant behavior whatever the location of the tumor is.
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DOI：
10.1097/PAI.0b013e3182392bb6
被引量：
年份：
2013
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