Nonepithelial malignant tumors of the pancreas are extremely rare neop
lasms with a frequency of similar to 0,6%. They are always explored be
cause of a suspected diagnosis of pancreatic carcinoma. Amongst the mo
re than 600 primary pancreatic neoplasms in our pancreatic tumor archi
ve only 5 neoplasms were of nonepithelial origin (one was a malignant
peripheral nerve sheath tumor [MPNST], one a leiomyosarcoma, one a mal
ignant mesothelioma, and two were peripheral neuroectodermal tumors [P
NET]. The differential diagnosis includes secondary infiltration of th
e pancreas by mesenchymal tumors of the retroperitoneum, undifferentia
ted pancreatic carcinoma and, especially in the case of PNET, malignan
t lymphoma. Preoperative chemotherapy and down-staging can improve the
operability and prognosis, especially in PNET.