title: Appendiceal mixed adenoneuroendocrine carcinomas, a rare entity that can present as a Krukenberg tumor: case report and review of the literature.
abstract: BACKGROUND
  Mixed adenoneuroendocrine carcinoma is a rare tumor recently recognized as a new category in the last World Health Organization (WHO) classification of appendiceal tumors (2010). This term has been proposed to designate carcinomas of the appendix that arise by progression from a pre-existing goblet cell carcinoid. Mixed adenoneuroendocrine carcinomas are more aggressive tumors than typical goblet cell carcinoids and usually present with peritoneal spreading and ovarian masses. Staging, some histological features, and completeness of surgery are factors that determine its evolution.
  
  
  METHODS
  We report the case of a mixed adenoneuroendocrine carcinoma--signet ring cell subtype--that presented as a Krukenberg tumor of unknown primary.
  
  
  CONCLUSIONS
  The review of literature is focused on the most recent WHO pathologic classification of appendiceal tumors containing goblet cell clusters, which seems to correlate with prognosis. A management proposal for mixed adenoneuroendocrine carcinomas reported in previous literature is also discussed. This ranges from right hemicolectomy to cytoreduction plus hyperthermic intraperitoneal chemotherapy, in both cases usually followed by intravenous chemotherapy.
authors.affiliation: Medical Oncology Department, Institut Català d'Oncologia Badalona (Hospital Germans Trias i Pujol), Carretera del Canyet s/n, 08916, Badalona, Barcelona, Spain. mromeo@iconcologia.net.
authors.forename: Margarita
authors.initials: M
authors.lastname: Romeo
authors.affiliation: Pathology Department, Hospital Germans Trias i Pujol, Carretera del Canyet s/n, 08916, Badalona, Barcelona, Spain. ariadnaqs@gmail.com.
authors.forename: Ariadna
authors.initials: A
authors.lastname: Quer
authors.affiliation: Gynecology Department, Gynecologic Oncology Section, Hospital Germans Trias i Pujol, Carretera del Canyet s/n, 08916, Badalona, Barcelona, Spain. atarrats.germanstrias@gencat.cat.
authors.forename: Antoni
authors.initials: A
authors.lastname: Tarrats
authors.affiliation: Gynecology Department, Hospital de l'Esperit Sant, Av. Mossèn Pons i Rabadà, s/n, 08923, Santa Coloma de Gramanet, Barcelona, Spain. cmolina@hes.scs.es.
authors.forename: Carlos
authors.initials: C
authors.lastname: Molina
authors.affiliation: King's College London, 16 De Crespigny Park, SE5 8AF, London, UK. joaquim.radua@kcl.ac.uk.|FIDMAG Germanes Hospitalàries-CIBERSAM, Dr. Antoni Pujadas, 38, 08830, Sant Boi de Llobregat, Barcelona, Spain. joaquim.radua@kcl.ac.uk.
authors.forename: Joaquim
authors.initials: J
authors.lastname: Radua
authors.affiliation: Medical Oncology Department, Institut Català d'Oncologia Badalona (Hospital Germans Trias i Pujol), Carretera del Canyet s/n, 08916, Badalona, Barcelona, Spain. jmanzano@iconcologia.net.
authors.forename: José-Luís
authors.initials: JL
authors.lastname: Manzano
mesh_terms: D000230:Adenocarcinoma / Q000175:diagnosis* / Q000601:surgery; D000368:Aged; D001063:Appendiceal Neoplasms / Q000175:diagnosis* / Q000601:surgery; D018278:Carcinoma, Neuroendocrine / Q000175:diagnosis* / Q000601:surgery; D018279:Carcinoma, Signet Ring Cell / Q000175:diagnosis* / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007725:Krukenberg Tumor / Q000175:diagnosis* / Q000601:surgery; D010051:Ovarian Neoplasms / Q000175:diagnosis* / Q000601:surgery; D011379:Prognosis