Key result
Tumor resection with cholecystectomy for a rare undifferentiated pleomorphic sarcoma originating in the porta hepatis resulted in the patient doing well without tumor recurrence at 1 year.
Case Report (n=1)
This is the first reported case of undifferentiated pleomorphic sarcoma originating in the porta hepatis, successfully treated with surgical resection.
UPS in porta hepatis expands hilar mass differential; leaves optimal management and prognosis open.
Undifferentiated pleomorphic sarcoma (UPS) is a soft tissue sarcoma in adults. Although it can occur in any part of the body, its most common site is the extremities, followed by the trunk and retroperitoneum. Here, we describe a rare case of UPS originating in the porta hepatis of an 83-year-old man that was incidentally detected via contrast-enhanced computed tomography. Because malignancy was suspected, we performed a tumor resection with cholecystectomy. Consistent with a diagnosis of UPS, histopathology revealed highly cellular lesions with scattered pleomorphic spindle cell fascicles arranged in a storiform pattern; further, results of tests for all immunohistochemical markers were negative. One year after surgery, the patient is currently doing well without any evidence of tumor recurrence. To our knowledge, this is the first report of UPS originating in the porta hepatis.
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Okimoto et al. (2017) conducted a case report in Undifferentiated pleomorphic sarcoma (UPS) originating in the porta hepatis (n=1). Tumor resection with cholecystectomy was evaluated on Tumor recurrence. Tumor resection with cholecystectomy for a rare undifferentiated pleomorphic sarcoma originating in the porta hepatis resulted in the patient doing well without tumor recurrence at 1 year.
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