title: Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential.
abstract: Solid-pseudopapillary tumors are uncommon neoplasms of low malignant potential generally occurring in young women. They often cause few symptoms and may reach a large size by the time they are detected. Degenerative cystic changes are common, and the clinical presentation is often that of a cystic pancreatic tumor. Pathological features include solid, cellular, hypervascular regions without gland formation, and degenerative pseudopapillae. The cells contain eosinophilic granules rich in alpha-1-antitrypsin and the nuclei are typically grooved. Despite its characteristic microscopic appearance, the immunophenotype (positive for vimentin, alpha-1-antitrypsin, and neuron specific enolase) is not specific and does not define a line of differentiation corresponding to any normal pancreatic cell type. Ultrastructural studies have also failed to identify specific differentiated features. Nonetheless, the biological behavior of solid-pseudopapillary tumor is well established. The tumor is indolent, with infrequent metastases to liver or peritoneum and usually long survival, even in the presence of disseminated disease.
authors.affiliation: Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA. klimstrd@mskcc.org
authors.forename: D S
authors.initials: DS
authors.lastname: Klimstra
authors.forename: B M
authors.initials: BM
authors.lastname: Wenig
authors.forename: C S
authors.initials: CS
authors.lastname: Heffess
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis; D002291:Carcinoma, Papillary / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D010190:Pancreatic Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure
chemical_list: D014408:Biomarkers, Tumor