The malignant nature of papillary and cystic neoplasm of the pancreas.

Two new cases of papillary and cystic neoplasm of the pancreas are reported. One patient was a 20-year-old woman with massive unresectable liver metastases, and the other was a 15-year-old boy. To study the natural history and malignant potential of this tumor, the English literature was reviewed to obtain an additional 56 cases. Clinical characteristics include pain and a mass in most patients, although many are found incidentally. Jaundice, hemoperitoneum, nausea, and vomiting are unusual findings. Most patients are treated by wide resection with good results. These tumors appear to be indolent. However, 16% of patients had major organ or blood vessel invasion, and 7% had liver metastases at some time during the course of their disease, illustrating the malignant nature of this tumor. Long-term follow-up is necessary to evaluate the efficacy of treatment, especially in the case of locally advanced and metastatic disease.