International Journal of Colorectal Disease

, Volume 28, Issue 2, pp 157–164 | Cite as

Colon carcinoma in childhood: review of the literature with four case reports

  • Gangmi Kim
  • Seung Hyuk Baik
  • Kang Young Lee
  • Hyuk Hur
  • Byung Soh Min
  • Chuhl Joo Lyu
  • Nam Kyu KimEmail author



Colorectal cancer (CRC) in childhood is extremely rare, and only a few case reports exist. We hereby present four cases of childhood CRC along with a literature review.


A retrospective review of four cases from our institute was conducted, and 15 articles from PubMed describing childhood CRC were reviewed.


Case reports: Four patients ranging in age from 11 to 14 years were treated for colon cancer between the years 2000 and 2011. The presenting symptoms varied and included abdominal pain, nausea, vomiting, diarrhea, constipation, and hematochezia, with symptom duration ranging from a few days to 4 months. No patient had a predisposing disease or syndrome. All patients underwent surgical resections, with the most prevalent histology being mucinous adenocarcinoma. Despite radical surgery followed by systemic chemotherapy, the disease progressed in all patients. Literature review: A total of 239 cases were reviewed. The youngest patient was 6 years old. The most frequent predisposing conditions were hereditary nonpolyposis colorectal cancer and familial adenomatous polyposis. Most of the patients presented advanced stage, with the most common histopathologic diagnosis being mucinous adenocarcinoma, followed by poorly differentiated adenocarcinoma and signet ring cell carcinoma. Even though most of the patients underwent aggressive treatment, the overall prognosis was poor.


Childhood CRC has an aggressive pathology and distinct genetic features, which result in an advanced stage at diagnosis and consequently a poor prognosis. Although the incidence is very low, every physician should be aware of CRC as a possible childhood diagnosis.


Colon carcinoma Rectal carcinoma Childhood 


  1. 1.
    Parkin DM (2001) Global cancer statistics in the year 2000. Lancet Oncol 2:533–543PubMedCrossRefGoogle Scholar
  2. 2.
    Pappo AS, Furman WL (2006) Management of infrequent cancers of childhood. In: Pizzo PA, Poplack DG (eds) Principles and practice of pediatric oncology, 5th edn. Lippincott Williams & Wilkins, Philadelphia, pp 1172–1201Google Scholar
  3. 3.
    Hill DA, Furman WL, Billups CA, Riedley SE, Cain AM, Rao BN, Pratt CB, Spunt SL (2007) Colorectal carcinoma in childhood and adolescence: a clinicopathologic review. J Clin Oncol 25:5808–5814PubMedCrossRefGoogle Scholar
  4. 4.
    Durno C, Aronson M, Bapat B, Cohen Z, Gallinger S (2005) Family history and molecular features of children, adolescents, and young adults with colorectal carcinoma. Gut 54:1146–1150PubMedCrossRefGoogle Scholar
  5. 5.
    Salas-Valverde S, Lizano A, Gamboa Y, Vega S, Barrantes M, Santamaria S, Zamora JB (2009) Colon carcinoma in children and adolescents: prognostic factors and outcome-a review of 11 cases. Pediatr Surg Int 25:1073–1076PubMedCrossRefGoogle Scholar
  6. 6.
    Ferrari A, Rognone A, Casanova M, Zaffignani E, Piva L, Collini P, Bertario L, Sala P, Leo E, Belli F, Gallino G (2008) Colorectal carcinoma in children and adolescents: the experience of the Istituto Nazionale Tumori of Milan, Italy. Pediatr Blood Cancer 50:588–593PubMedCrossRefGoogle Scholar
  7. 7.
    Radhakrishnan CN, Bruce J (2003) Colorectal cancers in children without any predisposing factors. A report of eight cases and review of the literature. Eur J Pediatr Surg 13:66–68PubMedCrossRefGoogle Scholar
  8. 8.
    Karnak I, Ciftci AO, Senocak ME, Buyukpamkcu N (1999) Colorectal carcinoma in children. J Pediatr Surg 34:1499–1504PubMedCrossRefGoogle Scholar
  9. 9.
    Andersson A, Bergdahl L (1976) Carcinoma of the colon in children: a repor of six new cases and a review of the literature. J Pediatr Surg 11:967–971PubMedCrossRefGoogle Scholar
  10. 10.
    Agrawal P, Rajwanshi A, Gupta N, Rao KL (2012) Mucinous adenocarcinoma of rectum in an 11-year-old boy: a case report. Diagn Cytopathol 40:252–254PubMedCrossRefGoogle Scholar
  11. 11.
    Angelini C, Crippa S, Uggeri F, Bonardi C, Sartori P (2005) Colorectal cancer with neuroendocrine differentiation in a child. Pediatr Surg Int 21:839–840PubMedCrossRefGoogle Scholar
  12. 12.
    Krishnamurthy S, Nirupam N, Seth A, Agarwal AK, Kumar P, Aneja S (2011) Adenocarcinoma of the rectum in a 13-year-old girl with Turner syndrome. J Pediatr Hematol Oncol 33:320–322PubMedCrossRefGoogle Scholar
  13. 13.
    Malik R, Kamath N (2011) Anorectal mucinous adenocarcinoma in child: a case report. Eur J Pediatr 170:1461–1463PubMedCrossRefGoogle Scholar
  14. 14.
    Muccillo A, Marchiori E, Penna CR, Guimaraes RR, Zanetti G, Abdalla G, Ventura N, Constantino CL, Pereira ML, Brandao V, Martins P, Canellas R, de OR V (2010) Colon mucinous adenocarcinoma in childhood: a case report with emphasis on image findings. Case report Med 2010:327634PubMedGoogle Scholar
  15. 15.
    Siqueira MA, Hayashi T, Yoshinaga K, Saisho S, Utsunomiya J, Sugihara K (2003) Colon cancer in a 14-year-old female with Turner syndrome. Dis Colon Rectum 46:1560–1562PubMedCrossRefGoogle Scholar
  16. 16.
    Yamamoto K, Tanaka T, Kuno K, Amoh Y, Takahashi Y, Murakami H (1994) Carcinoma of the colon in children: case repor and review of the Japanese literature. J Gastroenterol 29:647–652PubMedCrossRefGoogle Scholar
  17. 17.
    Taguchi T, Suita S, Hirata Y, Ishii E, Ueda K (1991) Carcinoma of the colon in children: a case report and review of 41 Japanese cases. J Pediatr Gastroenterol Nutr 12:394–399PubMedCrossRefGoogle Scholar
  18. 18.
    Bethel CAI, Bhattacharra N, Hutchinson C, Ruymann F, Cooney DR (1997) Alimentary tract malignancies in children. J Pediatr Surg 32:1004–1009PubMedCrossRefGoogle Scholar
  19. 19.
    Ladd AP, Grosfeld JL (2006) Gastrointestinal tumors in children and adolescents. Semin Pediatr Surg 15:37–47PubMedCrossRefGoogle Scholar
  20. 20.
    Sultan I, Rodriguez-Galindo C, El-Taani H, Pastore G, Casanova M, Gallino G, Ferrari A (2010) Distinct features of colorectal cancer in children and adolescents: a population-based study of 159 cases. Cancer 116:758–765PubMedCrossRefGoogle Scholar
  21. 21.
    Devroede GJ, Taylor WF, Sauer WG, Jackman RJ, Stickler GB (1971) Cancer risk and life expectancy of children with ulcerative colitis. N Engl J Med 285:17–21PubMedCrossRefGoogle Scholar
  22. 22.
    Kottmeier PK, Clatworthy HWJ (1965) Intestinal polyps and associated carcinoma in childhood. Am J Surg 110:709–716PubMedCrossRefGoogle Scholar
  23. 23.
    Ruo L, Cellini C, Jorge Puig La-Calle J, Murray M, Thaler HT, Quan SHQ, Guillem JG (2001) Limitations of family cancer history assessment at initial surgical consultation. Dis Colon Rectum 44:98–104PubMedCrossRefGoogle Scholar
  24. 24.
    Church J, McGannon E (2000) Family history of colorectal cancer. How often and how accurately is it recorded? Dis Colon Rectum 43:1540–1544PubMedCrossRefGoogle Scholar
  25. 25.
    Katballe N, Juul S, Christensen M, Orntoft TF, Wikman FP, Laurberg S (2001) Patient accuracy of reporting on hereditary non-polyposis colorectal cancer-related malignancy in family members. Br J Surg 88:1228–1233PubMedCrossRefGoogle Scholar
  26. 26.
    Goodenberger M, Lindor NM (2011) Lynch syndrome and MYH-associated polyposis. Review and testing strategy. J Clin Gastroenterol 45:488–500PubMedCrossRefGoogle Scholar
  27. 27.
    Hampel H, Frankel WL, Martin E, Arnold M, Khanduja K, Kuebler P, Clendenning M, Sotamaa K, Prior T, Westman JA, Panescu J, Fix D, Lockman J, LaJeunesse J, Comeras I, de la Chapelle A (2008) Feasibility of screening for Lynch syndrome among patients with colorectal cancer. J Clin Oncol 26:5783–5788PubMedCrossRefGoogle Scholar
  28. 28.
    Peltomaki P, Vasen H (2004) Mutations associated with HNPCC predisposition—update of ICG-HNPCC/INSiGHT mutation database. Dis Markers 20:269–276PubMedGoogle Scholar
  29. 29.
    Senter L (2012) Genetic testing by cancer site: colon (nonpolyposis syndromes). Cancer J 18:334–337PubMedCrossRefGoogle Scholar
  30. 30.
    Shi C, Washington K (2012) Molecular testing in colorectal cancer: diagnosis of Lynch syndrome and personalized cancer medicine. Am J Clin Pathol 137:847–859PubMedCrossRefGoogle Scholar
  31. 31.
    Worthley DL, Leggett BA (2010) Colorectal cancer: molecular features and clinical opportunities. Clin Biolchem Rev 31:31–38Google Scholar
  32. 32.
    Pritchard CC, Grady WM (2011) Colorectal cancer molecular biology moves into clinical practice. Gut 60:116–129PubMedCrossRefGoogle Scholar
  33. 33.
    Jasperson KW, Tuohy TM, Neklason DW, Burt RW (2010) Hereditary and familial colon cancer. Gastroenterology 138:2044–2058PubMedCrossRefGoogle Scholar
  34. 34.
    Vasen HF, van der Meulen-de Jong AE, de Vos Tot Nederveen Cappel WH, Oliveira J (2009) Familial colorectal cancer risk: ESMO clinical recommendations. Ann Oncol 20(Suppl 4):51–53PubMedGoogle Scholar
  35. 35.
    Poley JW, Wagner A, Hoogmans MM, Menko FH, Tops C, Kros JM, Reddingius RE, Meijers-Heijboer H, Kuipers EJ, Dinjens WN (2007) Biallelic germline mutations of mismatch-repair genes: a possible cause for multiple pediatric malignancies. Cancer 109:2349–2356PubMedCrossRefGoogle Scholar
  36. 36.
    Leenen CH, Geurts-Giele WR, Dubbink HJ, Reddingius R, van den Ouweland AM, Tops CM, van de Klift HM, Kuipers EJ, van Leerdam ME, Dinjens WN, Wagner A (2011) Pitfalls in molecular analysis for mismatch repair deficiency in a family with biallelic pms2 germline mutations. Clin Genet 80:558–565PubMedCrossRefGoogle Scholar
  37. 37.
    Herkert JC, Niessen RC, Olderode-Berends MJ, Veenstra-Knol HE, Vos YJ, van der Klift HM, Scheenstra R, Tops CM, Karrenbeld A, Peters FT, Hofstra RM, Kleibeuker JH, Sijmons RH (2011) Paediatric intestinal cancer and polyposis due to bi-allelic PMS2 mutations: case series, review and follow-up guidelines. Eur J Cancer 47:965–982PubMedCrossRefGoogle Scholar
  38. 38.
    Umar A, Boland CR, Terdiman JP, Syngal S, Adl C, Ruschoff J, Fishel R, Lindor NM, Burgart LJ, Hamelin R, Hamilton SR, Hiatt RA, Jass J, Lindblom A, Lynch HT, Peltomaki P, Ramsey SD, Rodriguez-Bigas MA, Vasen HFA, Hawk ET, Barrett JC, Freedman AN, Srivastava S (2004) Revised Bethesda guidelines for hereditary nonpolyposis colorectal cancer (Lynch syndrome) and microsatellite instability. J Natl Cancer Inst 96:261–268PubMedCrossRefGoogle Scholar
  39. 39.
    Chung MY, Park YS, Ryu SR, Ahn SB, Kim SH, Jo YJ, Han JK, Joo JE (2012) A case of colonic mucinous adenocarcinoma in 19-year-old male patient. Clin Endosc 45:103–107PubMedCrossRefGoogle Scholar
  40. 40.
    Nozoe T, Anai H, Nasu S, Sugimachi K (2000) Clinicopathological characteristics of mucinous carcinoma of the colon and rectum. J Surg Oncol 75:103–107PubMedCrossRefGoogle Scholar
  41. 41.
    Consorti F, Lorenzotti A, Midiri G, Md P (2000) Prognostic significance of mucinous carcinoma of colon and rectum: a prospective case–control study. J Surg Oncol 73:70–74PubMedCrossRefGoogle Scholar
  42. 42.
    Secco GB, Fardelli R, Campora E, Lapertosa G, Gentile R, Zoli S, Prior C (1994) Primary mucinous adenocarcinomas and signet-ring cell carcinomas of colon and rectum. Oncology 51:30–34PubMedCrossRefGoogle Scholar
  43. 43.
    Middelkamp JN, Haffner H (1963) Carcinoma of the colon in children. Pediatrics 32:558–571PubMedGoogle Scholar
  44. 44.
    Arai T, Kasahara I, Sawabe M, Kanazawa N, Kuroiwa K, Honma N, Aida J, Takubo K (2007) Microsatellite-unstable mucinous colorectal carcinoma occurring in the elderly: comparison with medullary type poorly differentiated adenocarcinoma. Pathol Int 57:205–212PubMedCrossRefGoogle Scholar
  45. 45.
    Ogino S, Brahmandam M, Cantor M, Namgyal C, Kawasaki T, Kirkner G, Meyerhardt JA, Loda M, Fuchs CS (2006) Distinct molecular features of colorectal carcinoma with signet ring cell component and colorectal carcinoma with mucinous component. Mod Pathol 19:59–68PubMedCrossRefGoogle Scholar
  46. 46.
    Tanaka H, Deng G, Matsuzaki K, Kakar S, Kim GE, Miura S, Sleisenger MH, Kim YS (2006) BRAF mutation, CpG island methylator phenotype and microsatellite instability occur more frequently and concordantly in mucinous than non-mucinous colorectal cancer. Int J Cancer 118:2765–2771PubMedCrossRefGoogle Scholar
  47. 47.
    Yoshitake N, Fujii S, Mukawa K, Tominaga K, Fukui H, Ichikawa K, Tomita S, Ono Y, Imai Y, Terano A, Hiraishi H, Fujimori T (2007) Mutational analysis of the BRAF gene in colorectal mucinous carcinoma in association with histological configuration. Oncol Rep 17:9–15PubMedGoogle Scholar
  48. 48.
    Ishizu H, Kumagai J, Eishi Y, Takizawa T, Koike M (2004) Mucin core protein expression by colorectal mucinous carcinomas with or without mucus hyperplasia. J Gastroenterol 39:125–132PubMedCrossRefGoogle Scholar
  49. 49.
    Kim DH, Kim JW, Cho JH, Baek SH, Kakar S, Kim GE, Sleisenger MH, Kim YS (2005) Expression of mucin core proteins, trefoil factors, APC and p21 in subsets of colorectal polyps and cancers suggests a distinct pathway of pathogenesis of mucinous carcinoma of the colorectum. Int J Oncol 27:957–964Google Scholar
  50. 50.
    Kim HS, Kang SH, Park CH, Yang WI, Jeung HC, Chung HC, Roh JK, Ahn JB, Kim NK, Min BS, Rha SY (2011) Genome-wide molecular characterization of mucinous colorectal adenocarcinoma using cDNA microarray analysis. Oncol Rep 25(3):717–727PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  • Gangmi Kim
    • 1
  • Seung Hyuk Baik
    • 1
  • Kang Young Lee
    • 1
  • Hyuk Hur
    • 1
  • Byung Soh Min
    • 1
  • Chuhl Joo Lyu
    • 2
  • Nam Kyu Kim
    • 1
    Email author
  1. 1.Department of SurgeryYonsei University College of MedicineSeoulKorea
  2. 2.Department of PediatricsYonsei University College of MedicineSeoulKorea

Personalised recommendations