title: Cervical neuroendocrine tumor in a young female with Lynch Syndrome.
abstract: Neuroendocrine tumors rarely occur in the cervix or other components of the reproductive system. These tumors have been associated with microsatellite instability, are very aggressive and often associated with poor outcome. Lynch syndrome is an inherited cancer syndrome that has also been associated with microsatellite instability. Here we report a 34-year-old female with Lynch syndrome and a family history of loss of DNA mismatch of the hereditary non-polyposis colorectal cancer repair gene expression who presented with a neuroendocrine tumor of her cervix as the first manifestation of Lynch syndrome. This is the first case reported of a neuroendocrine tumor of the cervix in a patient with Lynch syndrome. We also review the relationship between Lynch Syndrome and neuroendocrine tumors.
authors.affiliation: Department of Medicine, Internal Medicine, University of Missouri, Columbia, MO, USA.
authors.forename: Ibraheem
authors.initials: I
authors.lastname: Yousef
authors.affiliation: Diabetes and Cardiovascular Center, University of Missouri, Columbia, MO, USA.
authors.forename: Fadi
authors.initials: F
authors.lastname: Siyam
authors.affiliation: Department of Pathology, University of Missouri, Columbia, MO, USA.
authors.forename: Lester
authors.initials: L
authors.lastname: Layfield
authors.affiliation: Division of Hematology and Oncology, University of Missouri, Columbia, MO, USA.
authors.forename: Carl
authors.initials: C
authors.lastname: Freter
authors.affiliation: Department of Medicine, Internal Medicine, University of Missouri, Columbia, MO, USA.
authors.forename: James R
authors.initials: JR
authors.lastname: Sowers
mesh_terms: D048868:Adaptor Proteins, Signal Transducing / Q000235:genetics; D000328:Adult; D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000150:complications* / Q000235:genetics / Q000473:pathology; D053843:DNA Mismatch Repair / Q000235:genetics; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D000070957:MutL Protein Homolog 1; D018358:Neuroendocrine Tumors / Q000209:etiology* / Q000473:pathology; D009687:Nuclear Proteins / Q000235:genetics; D002583:Uterine Cervical Neoplasms / Q000209:etiology* / Q000473:pathology
chemical_list: D048868:Adaptor Proteins, Signal Transducing; C501486:MLH1 protein, human; D009687:Nuclear Proteins; D000070957:MutL Protein Homolog 1