Solid Pseudopapillary Neoplasms of the Pancreas: A Review

Solid Pseudopapillary Neoplasms (SPN) of the pancreas is rare tumors of low malignant potential composed of solid and cystic pseudopapillae which predominantly affect young females [1]. The first documentation of this lesion was by Lichtenstein in 1933, who described a young female presenting with a large abdominal tumor from which she ultimately died. At necropsy, she was found to have a large encapsulated cystic tumor of the pancreatic tail in addition to metastases to the liver, omentum, and peritoneum [2-5]. The patient was diagnosed as having a papillary cystadenocarcinoma; however, recent pathological review equates the findings most consistent with a mucinous cystic neoplasm. The first indisputable description of an SPN is attributed to Frantz, who in 1959 described a papillary tumor of the pancreas with solid and cystic components [6]. After this report, SPNs were described in several other case reports under a variety of names descriptive of its assorted characteristics, including “papillary epithelial neoplasia,” “Frantz tumor,” “papillary-cystic neoplasm,” and “adenocarcinoma of the pancreas in childhood” [7-9]. It was not until 1996 that the World Health Organization specifically classified the disease as “solid pseudopapillary tumor of the pancreas,” combining its different characteristics under one diagnosis [10].