Sarcomas of the pancreas are exceedingly rare. Baylor et al reported a 0.1% incidence of pancreatic sarcoma after review 5000 cases of pancreatic cancer . Amongst pancreatic sarcomas leiomyosarcomas tend to occur relatively frequent . Epitheloid sarcomas also known as carcinosarcomas represent an even rarer subgroup with very few reported cases in the english literature. Darvishian and colleagues reported the 7th case in the English literature . Thus the incidence of carcinosarcoma as a single entity is expected to be far below 0.1%.
According to Feather et al sarcomas of the pancreas occur frequently in younger individuals. The pancreatic caput is most commonly involved followed by the tail and the body . These features tend to correspond with those in the case presented. The patient, 52 years of age, relatively young, was diagnosed with a sarcoma of the pancreatic caput.
Clinically patients present with colicky pain, nausea and vomiting. These findings are basically similar to those of other pancreatic pathologies and are thus unspecific to sarcomas. Gray and many others consider a painless jaundice as a sign of an advanced lesion . This was true for the case presented.
The diagnosis of pancreatic pathologies is usually confirmed using imaging techniques like ultrasound, computed tomography (Ct), magnetic resonance imaging (MRI), endoscopic retrograde cholangiopancreaticogrphy (ERCP) [6–8].
On initial presentation abdominal ultrasound and ERCP were not suspicious of a pancreatic malignancy as seen in figure 1. A pancreatic process was suspected on an intraoperative cholangiogramm as seen in figure 2. This was later confirmed on CT and ERCP. To what extend an early ct scan would have confirmed a malignancy remains unclear.
Surgical resection is the only possible cure for pancreatic malignancies. Radical resections are done for localized lesions. Advanced lesions warrant palliation. Potts et al proved the importance of a palliative gastric bypass in advanced stages .
In this case, the patient presented with an advanced tumor, so curative resection was not feasible. A palliative gastroenteric anastomosis was done. The common bile duct was not revised since a stent was placed during ERCP . A venous port system was implanted and the patient was sent in for chemotherapy.
Pancreatic cancers generally have a poor prognosis since they tend to be diagnosed in an advanced stage. Sarcomas of the pancreas tend to grow much more rapidly and are believed to be associated with an even worse prognosis .
In the case presented, the sarcoma could have grown within three month, i.e between initial presentation in November 2010 and diagnosis in January 2011. This would support the notion that sarcomas tend to grow rapidly. The patient was discharged from the surgical department in a good shape and chemotherapy with Gencitabine, 5- FU and folinic acid was initiated.