title: High-Grade Neuroendocrine Colorectal Carcinomas: A Retrospective Study of 100 Patients.
abstract: BACKGROUND
  Colorectal high-grade neuroendocrine carcinomas (HGNEC) are a rare but aggressive group of malignancies without standard management recommendations.
  
  
  METHODS
  We retrospectively reviewed the records of 100 consecutive patients with histologically confirmed colorectal HGNEC diagnosed at MD Anderson Cancer Center between 1991 and 2013.
  
  
  RESULTS
  In our cohort, most tumors (89%) were small cell carcinoma, and most (60%) involved the sigmoid or the anorectal regions. Sixty-four patients (64%) presented with metastatic disease at diagnosis. Striking epidemiological and clinical differences between those established in small cell lung cancer (SCLC) and our cohort were noted, including significantly lower rates of smoking and lower risk of bone, brain metastases. Over 30% of the tumors were found associated with an adenoma. Median overall survival (OS) of the cohort was 14.7 months, with 2-year and 5-year OS rates of 23% and 8%, respectively. In patients with localized disease, multimodality therapy was associated with a trend toward improved median OS (20.4 vs. 15.4 months; P = .08). Metastases at presentation (OS 20.63 vs. 8.7 months; localized vs metastatic disease at presentation; P < .001) and elevated lactate dehydrogenase levels were strongly associated with a worse outcome.
  
  
  CONCLUSIONS
  In comparison to SCLC, less than half of the patients with colorectal HGNEC have history of smoking; metastatic patterns are also different between the 2 cancers. Nevertheless, HGNEC also has an aggressive biology, with the rectum being the most common site of origin. For localized disease, a multimodality approach seems to be associated with better outcomes, while systemic chemotherapy is the mainstay of treatment for advanced disease.
authors.affiliation: Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Bruno
authors.initials: B
authors.lastname: Conte
authors.affiliation: Division of Hematology and Oncology, Medical College of Wisconsin, Milwaukee, WI.
authors.forename: Ben
authors.initials: B
authors.lastname: George
authors.affiliation: Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Michael
authors.initials: M
authors.lastname: Overman
authors.affiliation: Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Jeannelyn
authors.initials: J
authors.lastname: Estrella
authors.affiliation: Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Zhi-Qin
authors.initials: ZQ
authors.lastname: Jiang
authors.affiliation: Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Amir
authors.initials: A
authors.lastname: Mehrvarz Sarshekeh
authors.affiliation: Department of Thoracic/Head and Neck Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Renata
authors.initials: R
authors.lastname: Ferrarotto
authors.affiliation: Instituto do Câncer do Estado de São Paulo, Universidade de São Paulo, Sao Paulo, Brazil.
authors.forename: Paulo M
authors.initials: PM
authors.lastname: Hoff
authors.affiliation: Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Asif
authors.initials: A
authors.lastname: Rashid
authors.affiliation: Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: James C
authors.initials: JC
authors.lastname: Yao
authors.affiliation: Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Scott
authors.initials: S
authors.lastname: Kopetz
authors.affiliation: Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX. Electronic address: adasari@mdanderson.org.
authors.forename: Arvind
authors.initials: A
authors.lastname: Dasari
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018278:Carcinoma, Neuroendocrine / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D015179:Colorectal Neoplasms / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D012189:Retrospective Studies
keywords: Colon; Metastasis; Rectum; Small cell carcinoma; Smoking; Survival
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