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Colorectal Carcinoma in Childhood and Adolescence: A Clinicopathologic Review

D. Ashley Hill, Wayne L. Furman, Catherine A. Billups, Shannon E. Riedley, Alvida M. Cain, Bhaskar N. Rao, Charles B. Pratt, Sheri L. Spunt

From the Departments of Pathology, Hematology-Oncology, Biostatistics, and Surgery, St Jude Children's Research Hospital; and the Department of Pediatrics, The University of Tennessee College of Medicine, Memphis, TN
Deceased

Address reprint requests to D. Ashley Hill, MD, Lauren V. Ackerman Division of Surgical Pathology, Washington University Medical Center, Box 8118, 660 S Euclid Ave, St Louis, MO 63110; e-mail: hill{at}path.wustl.edu

Purpose Pediatric colorectal carcinoma (CRC) is rare, but the available data suggest that it is more likely than adult CRC to be advanced at presentation and to have a poor outcome. We sought to better characterize pediatric CRC.

Patients and Methods We reviewed the clinical and pathologic features, prognostic factors, and outcome of CRC in 77 children and adolescents (ages 7 to 19 years) referred to St Jude Children's Research Hospital between 1964 and 2003.

Results At presentation, 76 patients had one or more signs or symptoms of CRC (abdominal pain, altered bowel habits, weight loss, anemia). Tumors were evenly distributed between the right and left colon; 62% were mucinous adenocarcinoma. At presentation, 86% of patients had advanced-stage disease; more than half had distant metastases. Overall outcome was poor. Advanced stage and mucinous histology were significant predictors of adverse outcome. Stage-specific survival at 10 years was 67% ± 27% (stage 1), 38% ± 15% (stage 2), 28% ± 11% (stage III), and 7% ± 4% (stage 4). Although no patient had a diagnosis of polyposis syndrome before diagnosis of CRC, 17 (22%) had colon polyps and eight (including two who previously underwent pelvic radiotherapy) had multiple polyps.

Conclusion Initial signs and symptoms of CRC are similar in pediatric and adult patients. The strikingly higher frequency of mucinous histology suggests that the biology of CRC differs in pediatric and adult patients and may contribute to poor outcomes. Children should be included in prospective clinical trials for CRC.

Supported in part by Grants No. CA21765 and CA23099 from the National Institutes of Health and by the American Lebanese Syrian Associated Charities (ALSAC).

Presented in part at the Society for Pediatric Pathology companion meeting at the United States and Canadian Academy of Pathology Spring Meeting, March 25-26, 2000, New Orleans, LA, and at the 36th Annual Meeting of the American Society of Clinical Oncology, May 20-23, 2000, New Orleans, LA. A subset of these cases was described in 1985.

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

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