title: Pediatric Colorectal Carcinoma is Associated With Excellent Outcome in the Context of Cancer Predisposition Syndromes.
abstract: BACKGROUND
  Colorectal carcinoma (CRC) is the second most common adult cancer in Germany, however, it is extremely rare in children and adolescents. In these patients, previous literature describes aggressive behavior and diagnosis at advanced stage.
  
  
  METHODS
  Thirty-one patients with CRC age ≤ 18 years and treated between 1990 and 2012 have been identified through the structures and registries of the German Society for Pediatric Oncology and Hematology.
  
  
  RESULTS
  The age range was 9-18 years (median 13.5 years); the median follow-up time was 43.9 months (range 1-124 months). Twenty-six patients (84%) were tested for a genetic tumor syndrome (GTS); of these, 11 patients (35% of all patients) tested positive (eight cases of Lynch syndrome, one patient with familial adenomatous polyposis, two patients with constitutional mismatch repair deficiency). An unfavorable histology was reported in 55% of the records (n = 17), a poor differentiation (grade III) in 68% of carcinoma (n = 21). Overall survival (OS) and event-free survival at 5 years was 52.0% and 65.6%, respectively. Five-year survival according to stage was 100% in stage II (n = 2), 100% in stage III (n = 13), and 12.9% in stage IV (n = 15; P < 0.001). Five-year OS in patients with and without a defined GTS was 100% and 36.5% (P = 0.019), respectively.
  
  
  CONCLUSIONS
  Children and adolescents with CRC are frequently diagnosed in advanced stages and have an unfavorable prognosis. In this study, a high percentage of pediatric CRC patients presented with a tumor predisposition syndrome and showed an especially favorable OS.
authors.affiliation: Department of Pediatric Hematology and Oncology, University Children's Hospital Erlangen, Germany.
authors.forename: Marie L
authors.initials: ML
authors.lastname: Weber
authors.affiliation: Clinic of Pediatrics, Dortmund, Germany.
authors.forename: Dominik T
authors.initials: DT
authors.lastname: Schneider
authors.affiliation: Department of Pediatric Hematology and Oncology, University Children's Hospital Erlangen, Germany.
authors.forename: Sonja
authors.initials: S
authors.lastname: Offenmüller
authors.affiliation: German Childhood Cancer Registry (GCCR), Institute of Medical Biostatistics, Epidemiology and Informatics (IMBEI), University Medical Center Mainz, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Kaatsch
authors.affiliation: Department of Pediatric Oncology and Hematology, University Children's Hospital Greifswald, Germany.
authors.forename: Hagen Graf
authors.initials: HG
authors.lastname: Einsiedel
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Austria.
authors.forename: Martin
authors.initials: M
authors.lastname: Benesch
authors.affiliation: Pediatric Hematology and Oncology, University Children's Hospital Kiel, Germany.
authors.forename: Alexander
authors.initials: A
authors.lastname: Claviez
authors.affiliation: Pediatric Hematology and Oncology, University Children's Hospital Tuebingen, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Ebinger
authors.affiliation: Pediatric Hematology and Oncology, University Children's Hospital Goettingen, Germany.
authors.forename: Christof
authors.initials: C
authors.lastname: Kramm
authors.affiliation: Pediatric Hematology and Oncology, University Children's Hospital Hannover, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Kratz
authors.affiliation: Department of Pediatric Hematology and Oncology, University Children's Hospital Erlangen, Germany.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Lawlor
authors.affiliation: Department of Paidopathology, University of Kiel, Germany.
authors.forename: Ivo
authors.initials: I
authors.lastname: Leuschner
authors.affiliation: Clinical Tumor Registry, Department of Surgery, University of Erlangen, Germany.
authors.forename: Susanne
authors.initials: S
authors.lastname: Merkel
authors.affiliation: Department of Pediatric Hematology and Oncology, University Children's Hospital Erlangen, Germany.
authors.forename: Markus
authors.initials: M
authors.lastname: Metzler
authors.affiliation: Department of Pediatric Surgery, University Children's Hospital Hannover, Germany.
authors.forename: Rainer
authors.initials: R
authors.lastname: Nustede
authors.affiliation: Tumor Registry, University of Erlangen-Nuernberg, Germany.
authors.forename: Sabine
authors.initials: S
authors.lastname: Petsch
authors.affiliation: Pediatric Oncology and Hematology, Charité University Medicine, Berlin, Germany.
authors.forename: Karl-Heinz
authors.initials: KH
authors.lastname: Seeger
authors.affiliation: Department of Pediatric Hematology and Oncology, University Children's Hospital Wuerzburg, Germany.
authors.forename: Paul-Gerhardt
authors.initials: PG
authors.lastname: Schlegel
authors.affiliation: Department of Pediatric Hematology and Oncology, University Children's Hospital Dresden, Germany.
authors.forename: Meinolf
authors.initials: M
authors.lastname: Suttorp
authors.affiliation: Institute of Pharmacology of Natural Products and Clinical Pharmacology, University of Ulm, Germany.
authors.forename: Oliver
authors.initials: O
authors.lastname: Zolk
authors.affiliation: Department of Pediatric Hematology and Oncology, University Children's Hospital Erlangen, Germany.
authors.forename: Ines B
authors.initials: IB
authors.lastname: Brecht
mesh_terms: D000230:Adenocarcinoma / Q000235:genetics* / Q000401:mortality* / Q000473:pathology*; D000293:Adolescent; D002648:Child; D015179:Colorectal Neoplasms / Q000235:genetics* / Q000401:mortality* / Q000473:pathology*; D018572:Disease-Free Survival; D005260:Female; D020022:Genetic Predisposition to Disease; D005858:Germany; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D011379:Prognosis; D012042:Registries
keywords: Lynch syndrome; cancer predisposition; colorectal carcinoma; familial adenomatous polyposis; mismatch repair deficiency; pediatric rare tumors