title: P16-positive continuous minimal deviation adenocarcinoma and gastric type adenocarcinoma in a patient with Peutz-Jeghers syndrome.
abstract: We report a case of Peutz-Jeghers syndrome (PJS) in a 33-year-old female patient with synchronous uterine cervical minimal deviation adenocarcinoma (MDA) and gastric type adenocarcinoma (GTA). The patient was diagnosed with PJS at the age of 10. At the time of consultation, she complained of watery discharge. Magnetic resonance imaging of the pelvis showed a poorly circumscribed mass in the uterine cervix. Histologically, both MDA and GTA components, as well as their transitional area, were observed. Both components were diffusely positive for MUC6, CK7 and, robustly, for p16. Moreover, the components were negative for ER, PgR and CEA, while HIK1083 and CK20 positive cells were found focally. Ki-67 labeling index in the MDA component was 5% while that in the GTA component was 50%. This case of GTA accompanied by MDA in a patient with PJS is distinct from the single previously-reported comparable case of which we are aware, with respect to the overexpression of p16 protein, an event considered rare in these tumors, and the continuity between the MDA and GTA components. This continuity favors the hypothesis that GTA arises from the dedifferentiation of MDA.
authors.affiliation: Department of Integrated Diagnostic Pathology, Nippon Medical School Bunkyo-ku, Tokyo 113-8602, Japan.
authors.forename: Wei-Xia
authors.initials: WX
authors.lastname: Peng
authors.affiliation: Department of Integrated Diagnostic Pathology, Nippon Medical School Bunkyo-ku, Tokyo 113-8602, Japan.
authors.forename: Shoko
authors.initials: S
authors.lastname: Kure
authors.affiliation: Department of Integrated Diagnostic Pathology, Nippon Medical School Bunkyo-ku, Tokyo 113-8602, Japan.
authors.forename: Kousuke
authors.initials: K
authors.lastname: Ishino
authors.affiliation: Department of Obstetrics and Gynecology, Nippon Medical School Hospital Bunkyo-ku, Tokyo 113-8602, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Kurose
authors.affiliation: Department of Obstetrics and Gynecology, Nippon Medical School Hospital Bunkyo-ku, Tokyo 113-8602, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Yoneyama
authors.affiliation: Department of Integrated Diagnostic Pathology, Nippon Medical School Bunkyo-ku, Tokyo 113-8602, Japan.
authors.forename: Ryuichi
authors.initials: R
authors.lastname: Wada
authors.affiliation: Department of Integrated Diagnostic Pathology, Nippon Medical School Bunkyo-ku, Tokyo 113-8602, Japan.
authors.forename: Zenya
authors.initials: Z
authors.lastname: Naito
mesh_terms: D000230:Adenocarcinoma / Q000737:chemistry* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis*; D001706:Biopsy; D054337:Cell Dedifferentiation; D019941:Cyclin-Dependent Kinase Inhibitor p16; D005260:Female; D006801:Humans; D007044:Hysterectomy; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D009363:Neoplasm Proteins / Q000032:analysis*; D009378:Neoplasms, Multiple Primary / Q000737:chemistry* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D010580:Peutz-Jeghers Syndrome / Q000175:diagnosis / Q000235:genetics*; D011237:Predictive Value of Tests; D015854:Up-Regulation; D014594:Uterine Neoplasms / Q000737:chemistry* / Q000235:genetics / Q000473:pathology / Q000601:surgery
chemical_list: D014408:Biomarkers, Tumor; C000614131:CDKN2A protein, human; D019941:Cyclin-Dependent Kinase Inhibitor p16; D009363:Neoplasm Proteins
keywords: P16; Peutz-Jeghers syndrome; cervical adenocarcinoma; gastric type adenocarcinoma; immunohistochemistry; minimal deviation adenocarcinoma