Introduction Rhabdomyosarcoma (RMS) is a primary malignancy that arises from the embryonic mesenchyme with the potential to differentiate into skeletal muscle. RMS of the biliary tree is extremely rare. We report a case of an undifferentiated pleomorphic RMS involving the liver and pancreas. Presentation of case A 62 year old Caucasian woman with rapidly growing abdominal mass and a history of endometrial adenocarcinoma underwent laparotomy due to compression symptoms and concerns of malignancy. A large mass arising from the pancreas and extending into the liver was identified and resected with a distal pancreatectomy associated with a left lateral liver segmentectomy. A diagnosis of pleomorphic RMS was made from the pathology specimen. Chemotherapy and radiotherapy were also performed. Unfortunately the patient died 2 years following treatment due to recurrence of the disease. Discussion P-RMS in the biliary tree is extremely rare (0.5%) and mostly seen in infants and children. Preoperative diagnosis is challenging since the symptoms are unspecific. Preoperative imaging rarely contributes to the final diagnosis. The only possible treatment for adult RMS is surgical resection of the tumor followed by chemotherapy and radiotherapy. Long-term prognosis of P-RMS reported (predominantly of limbs) is poor. To our knowledge, no previous cases of RMS originating from the pancreas have been reported. Conclusion However RMS is an extremely rare tumor in adults, it should be included in the differential diagnosis of patients with atypical pancreatic and liver lesions.
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