Key result
Primary undifferentiated pleomorphic sarcoma of the pancreas is an extremely rare malignancy that typically presents as a solid cystic lesion on imaging and is primarily managed with radical surgical resection.
Why the study?
Primary undifferentiated pleomorphic sarcoma of the pancreas is exceedingly rare, clinicians have poor recognition of it, and its epidemiology, diagnosis, and treatment have not been established.
Case Report (n=16)
Primary undifferentiated pleomorphic sarcoma of the pancreas is an extremely rare malignancy with a poor prognosis, and this report provides the first detailed ultrasonography features of the disease.
BACKGROUND: Primary undifferentiated pleomorphic sarcoma (UPS) of the pancreas is an exceedingly rare malignant tumor, with only 15 cases have been reported in the medical literature. At present, clinicians have poor recognition of the tumor, the epidemiology, diagnosis, and treatment of this disease have yet not been established. CASE PRESENTATION: In this report, we depict the clinical and imaging characteristics of a 37-year-old man presenting with a primarily cystic UPS. The patient complained of epigastric pain and distention over 20 days. Abdominal CT and pancreatic magnetic resonance imaging revealed cystic and cystic solid masses in the pancreatic body and tail. An abdominal ultrasound echogram revealed the mass in the body of the pancreas to be cystic with separation echo inside, and the wall was thick, not smooth. Besides, a hypoechoic mass was seen in the tail area of the pancreas with an inhomogeneous echoic pattern, containing small patches of no echo zone in the central. Microscopically, spindle fibroblast-like cells are arranged in a characteristic storiform pattern with pleomorphic and multinucleated cells. Immunohistochemically, tumor cells were positive for CD68 and vimentin. Seven months postoperatively, he was diagnosed with pulmonary lymph node metastasis and died 5 months later. Combined with this case report, we also reviewed the literature regarding UPS of the pancreas. CONCLUSIONS: As we know, this is the first report on ultrasonography findings of pancreatic UPS. Despite there are no distinctive manifestation of UPS, a solid cystic lesion on ultrasonography or a hypodense area in the lesion on T2-weighted imaging, should be considered for differential diagnosis with pancreatic UPS. We believe this article may add some ideas into the diagnosis and therapy of patients with this tumor.
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Liang et al. (2022) conducted a case report in Primary undifferentiated pleomorphic sarcoma of the pancreas (n=16). Surgical resection was evaluated. Primary undifferentiated pleomorphic sarcoma of the pancreas is an extremely rare malignancy that typically presents as a solid cystic lesion on imaging and is primarily managed with radical surgical resection.
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