Abstract
A peripheral primitive neuroectodermal tumor (pPNET) is a small round cell tumor occurring mostly in children or young adults and categorized into the Ewing sarcoma family of tumors. pPNETs originating from the pancreas are especially rare, and only 25 cases have been reported in the literature. We report a case of a 22-year-old man who had a giant expansive tumor located in the uncinate process of the pancreas, 80 mm in diameter resulting in obstruction in the duodenum. The patient underwent a pancreaticoduodenectomy. The histological examination showed that the pancreatic tumor was composed of atypical small round cells. Immunohistochemical findings were positive for CD99. An Ewing sarcoma breakpoint region 1 gene 22q12 rearrangement was proven by a two-color fluorescence in situ hybridization assay. We diagnosed the tumor as a pPNET of the pancreas, which, according to the literature, is highly aggressive with poor prognosis. A multidisciplinary approach to treat these neoplasms should improve the prognoses.
Highlights
Peripheral primitive neuroectodermal tumors are primary malignant neoplasms, usually occurring in children or young adults. These neoplasms are small round cell tumors arising from primitive neuroepithelial stem cells and categorized into the Ewing sarcoma family of tumors (ESFTs), which display common characteristics of morphology, histology, and genetics [1]
We reported a case of an extremely rare tumor originating from the pancreatic head in a young adult
When examining a young patient suspected of a small round cell tumor or undifferentiated tumor, frozen samples of the tumor should be used for a definitive diagnosis
Summary
A peripheral primitive neuroectodermal tumor originating from the pancreas: a case report and review of the literature.
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