title: Systemic chemotherapy and surgical cytoreduction for poorly differentiated and signet ring cell adenocarcinomas of the appendix.
abstract: BACKGROUND
  Poorly differentiated and signet ring cell adenocarcinomas of the appendix represent a subset with aggressive tumor biology and poor outcomes with few studies evaluating the impact of systemic chemotherapy and cytoreductive surgery (CRS).
  
  
  METHODS
  A retrospective chart review of patients with either poorly differentiated and signet ring cell appendiceal adenocarcinomas was completed from 1992 to 2010.
  
  
  RESULTS
  One hundred forty-two patients were identified. Seventy-eight patients with metastatic disease received chemotherapy. Radiographic response was 44%, median progression-free survival (PFS) was 6.9 months, and median overall survival (OS) was 1.7 years. In multivariate analysis, response to chemotherapy [hazard ratio (HR) 0.5; P = 0.02] predicted improved PFS, and complete CRS (HR 0.3; P = 0.004) predicted improved OS. Patients who underwent complete CRS (n = 26) had a median relapse-free survival (RFS) of 1.2 years and a median OS of 4.2 years. In multivariate analysis for this subset, complete cytoreduction score of 0 was significantly correlated with improved RFS (HR 0.07; P = 0.01) and OS (HR 0.02; P = 0.01).
  
  
  CONCLUSIONS
  Systemic chemotherapy appears to be a viable treatment option for patients with metastatic poorly differentiated and signet ring cell appendiceal adenocarcinomas. Complete CRS is associated with improved RFS and OS, though part of this benefit likely reflects the selection of good tumor biology.
authors.affiliation: Departments of Gastrointestinal Medical Oncology.
authors.forename: C H
authors.initials: CH
authors.lastname: Lieu
authors.affiliation: Departments of Surgical Oncology.
authors.forename: L A
authors.initials: LA
authors.lastname: Lambert
authors.affiliation: Departments of Gastrointestinal Medical Oncology.
authors.forename: R A
authors.initials: RA
authors.lastname: Wolff
authors.affiliation: Departments of Gastrointestinal Medical Oncology.
authors.forename: C
authors.initials: C
authors.lastname: Eng
authors.affiliation: Departments of Biostatistics.
authors.forename: N
authors.initials: N
authors.lastname: Zhang
authors.affiliation: Departments of Biostatistics.
authors.forename: S
authors.initials: S
authors.lastname: Wen
authors.affiliation: Departments of Surgical Oncology.
authors.forename: S
authors.initials: S
authors.lastname: Rafeeq
authors.affiliation: Departments of Pathology, The University of Texas MD Anderson Cancer Center, Houston, USA.
authors.forename: M
authors.initials: M
authors.lastname: Taggart
authors.affiliation: Departments of Surgical Oncology.
authors.forename: K
authors.initials: K
authors.lastname: Fournier
authors.affiliation: Departments of Surgical Oncology.
authors.forename: R
authors.initials: R
authors.lastname: Royal
authors.affiliation: Departments of Surgical Oncology.
authors.forename: P
authors.initials: P
authors.lastname: Mansfield
authors.affiliation: Departments of Gastrointestinal Medical Oncology. Electronic address: moverman@mdanderson.org.
authors.forename: M J
authors.initials: MJ
authors.lastname: Overman
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D001063:Appendiceal Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D018279:Carcinoma, Signet Ring Cell / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies