Undifferentiated pleomorphic sarcoma of the pancreas: a rare case report and literature review

Abstract

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.

Primary Pancreatic Undifferentiated Pleomorphic Sarcoma

“…2 Multimodal treatment with chemotherapy, radiofrequency ablation, and surgery improves the prognosis and survival of patients with primary pancreatic UPS. 7 The chemotherapy regimen in our case was recommended based on histology and currently existing data on adjuvant chemotherapy in soft-tissue sarcoma. Primary pancreatic sarcomas are not common and given the heterogeneity of primary sarcoma location; decision making regarding systemic therapy is largely driven by histology.…”

Pancreatic panniculitis and polyarthropathy due to undifferentiated pleomorphic sarcoma

“…The most common presenting symptom of pancreatic UPS is epigastric pain, in addition to nausea, vomiting, weight loss, and abdominal mass. 8 , 9 Diagnostic workup for primary pancreatic UPS typically involves an abdominal computed tomography revealing a heterogeneous mass with calcifications and necrosis. 8 Histopathology evaluation is essential for diagnosing UPS and reveals atypical pleomorphic spindle cells.…”

“… 8 Histopathology evaluation is essential for diagnosing UPS and reveals atypical pleomorphic spindle cells. 8 , 9 , 10 Definitive diagnosis requires immunohistochemical exams to exclude other malignancies. 10 Radical resection is the mainstay of treatment for retroperitoneal sarcomas, including primary pancreatic UPS.…”

“…Adjuvant therapeutic options include radiotherapy and chemotherapy with doxorubicin and ifosfamide. 9 …”

Undifferentiated Pleomorphic Sarcoma of the Duodenal Papilla: A Rare Case and Worth Discussing History

“…Undifferentiated pleomorphic sarcoma (UPS) is the most common soft tissue sarcomas in the elderly ( 1 , 2 ). It is a neoplasm considered to originate from primitive mesenchymal cells, arising from soft tissue or bone, usually in the extremities or retroperitoneum ( 3 ).…”