Primary Retroperitoneal Mucinous Cystadenoma with Sarcomatous Carcinoma in Mural Nodule

Primary retroperitoneal mucinous cystic tumors are extremely rare neoplasms that usually occur in women. Their histogenesis has not been clarified. Their low incidence has meant that it difficult to define therapeutic strategies or prognosis, or to record survival data. We describe a 35 year-old female with a retroperitoneal mucinous cystadenoma presenting a mural nodule with foci of sarcomatoid carcinoma. The entire tumor was surgically removed. After surgery, the patient received six cycles of carboplatin and paclitaxel. After 30 months of follow up she has shown no signs of recurrence. We review the etiology, diagnosis and therapeutic options in these extremely rare tumors.