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.

Keywords: Case report; Pancreatic neoplasms; Treatment; Ultrasonography; Undifferentiated pleomorphic sarcoma.

Fig. 1

Image of pleomorphic sarcoma of the pancreas. A The abdominal CT scan showed two hypointense lesions in the pancreas body and tail. B MRI revealed two round cystic abnormal signal involving the body and tail of the pancreas. C, D Ultrasound imaging exhibited a cystic echo in the body of the pancreas with separation and hypoecho in the tail. (the red arrow indicates the masses)

Fig. 2

Microscopic section reveals spindle cells arranged in a storiform pattern, with polymorphic neoplastic cells (hematoxylin and eosin, × 40, × 100 original magnification) (a, b). The cells exhibit the diffuse positive reaction to vimentin (c). The cells are positive for p53 (d)