Key result
A 56-year-old male was diagnosed with primary hepatic undifferentiated pleomorphic sarcoma, a rare malignant mesenchymal tumor, after presenting with epigastric pain and weight loss.
Why the study?
Primary hepatic undifferentiated pleomorphic sarcoma is a rare malignant mesenchymal tumor with a nonspecific clinical and radiologic presentation that is often a diagnosis of exclusion.
Case Report (n=1)
No
Primary hepatic undifferentiated pleomorphic sarcoma is a rare malignant mesenchymal tumor that requires early identification to prevent distant metastasis.
May warrant considering primary hepatic UPS in atypical liver lesions; extends sparse case reports but leaves management open.
Primary hepatic undifferentiated pleomorphic sarcoma (UPS) is a rare malignant mesenchymal tumor with a nonspecific clinical and radiologic presentation. Primary hepatic UPS is often a diagnosis of exclusion made by multiple immunohistological testing that rules out hepatic, hematologic, neural, and epithelial origin. Stains for mesenchymal origin are usually the only positive stain and do not demonstrate evidence of specific mesenchymal cell differentiation. We report a case of a 56-year-old male with no significant past medical history that presented with complaint of epigastric abdominal pain of six months duration. A computed tomography (CT) scan of the abdomen and pelvis exhibited numerous hepatic masses involving right and left hepatic lobe. A CT-guided core needle biopsy discovered undifferentiated/pleomorphic sarcoma. Histomorphology showed spindle cell neoplasm without recognizable hepatic tissue. Immunohistochemistry (IHC) stains were positive for smooth muscle actin (SMA) but failed to establish a more specific histogenesis. Furthermore, IHC stains revealed spindle neoplastic cells with focal and patchy positive h-caldesmon (approximately 10-15% of neoplastic cells), and negative for desmin. Given these results, the diagnosis of undifferentiated/pleomorphic sarcoma was established. It is imperative to consider UPS in the differential diagnosis of large liver lesions without evidence of differentiation. Early identification of this rare tumor can prevent the possibility of distant metastasis and improve survival among patients.
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Pellegrini et al. (2022) conducted a case report in Primary hepatic undifferentiated pleomorphic sarcoma (n=1). Diagnosis of primary hepatic undifferentiated pleomorphic sarcoma was evaluated. A 56-year-old male was diagnosed with primary hepatic undifferentiated pleomorphic sarcoma, a rare malignant mesenchymal tumor, after presenting with epigastric pain and weight loss.
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