Marie L Weber1, Dominik T Schneider2, Sonja Offenmüller1, Peter Kaatsch3, Hagen Graf Einsiedel4, Martin Benesch5, Alexander Claviez6, Martin Ebinger7, Christof Kramm8, Christian Kratz9, Jennifer Lawlor1, Ivo Leuschner10, Susanne Merkel11, Markus Metzler1, Rainer Nustede12, Sabine Petsch13, Karl-Heinz Seeger14, Paul-Gerhardt Schlegel15, Meinolf Suttorp16, Oliver Zolk17, Ines B Brecht1. 1. Department of Pediatric Hematology and Oncology, University Children's Hospital Erlangen, Germany. 2. Clinic of Pediatrics, Dortmund, Germany. 3. German Childhood Cancer Registry (GCCR), Institute of Medical Biostatistics, Epidemiology and Informatics (IMBEI), University Medical Center Mainz, Germany. 4. Department of Pediatric Oncology and Hematology, University Children's Hospital Greifswald, Germany. 5. Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Austria. 6. Pediatric Hematology and Oncology, University Children's Hospital Kiel, Germany. 7. Pediatric Hematology and Oncology, University Children's Hospital Tuebingen, Germany. 8. Pediatric Hematology and Oncology, University Children's Hospital Goettingen, Germany. 9. Pediatric Hematology and Oncology, University Children's Hospital Hannover, Germany. 10. Department of Paidopathology, University of Kiel, Germany. 11. Clinical Tumor Registry, Department of Surgery, University of Erlangen, Germany. 12. Department of Pediatric Surgery, University Children's Hospital Hannover, Germany. 13. Tumor Registry, University of Erlangen-Nuernberg, Germany. 14. Pediatric Oncology and Hematology, Charité University Medicine, Berlin, Germany. 15. Department of Pediatric Hematology and Oncology, University Children's Hospital Wuerzburg, Germany. 16. Department of Pediatric Hematology and Oncology, University Children's Hospital Dresden, Germany. 17. Institute of Pharmacology of Natural Products and Clinical Pharmacology, University of Ulm, Germany.
Abstract
INTRODUCTION: 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. METHOD: 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. CONCLUSION: 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.
INTRODUCTION: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. METHOD: 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. CONCLUSION: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.