Cancer Res Treat.  2020 Jan;52(1):117-127. 10.4143/crt.2019.092.

Pediatric Adenocarcinoma in Korea: A Multicenter Study

• Yang HB
• Namgoong J
• Kim KH
• Kim DY
• Park J
• Shin HB
• Youn JK
• Lee S
• Lee JW
• Jung SE
• Chung JH
• Choe YM
• Heo TG
• Ho IG
• Kim HY

• KMID: 2501207
• DOI: http://doi.org/10.4143/crt.2019.092

Abstract

Purpose
Adenocarcinoma is an extremely rare malignancy in the pediatric population. Research regarding pediatric adenocarcinoma is very rare in Korea. This study aimed to investigate the clinical features of pediatric adenocarcinomas of various primary organ sites in Korea.
Materials and Methods
Pediatric patients under 18 years, diagnosed with adenocarcinoma of various sites between January 1995 and December 2016, were included. We retrospectively reviewed patient and tumor characteristics and calculated survival estimates, reported as 5-year survival rate and 95% confidence interval.
Results
Of 80 patients (median age, 15 years; range, 10 to 17 years), 37 (46.3%) were men, and 24 (30%) had a family history of cancer or underlying disease relevant to malignancy. The cancer locations were the colon and rectum (n=32), ovaries (n=18), stomach (n=15), lung (n=4), small bowel (n=1), and other sites (n=10). Totally, 54.8% patients (42/77) had stage 3 or 4 disease. The median follow-up period was 2.0 years (range, 0 to 20.4). The 5-year overall survival estimate for all patients, and for those with stomach, colorectal, ovarian, and other cancer sites were 57.9%±11.5%, 58.2%±25.7%, 41.5%±18.2%, 87.5%±16.2%, and 64.0%±34.4%, respectively. The 5-year survival rate differed significantly between categories of adenocarcinomas into gastrointestinal (GI) (44.7%) and non-GI adenocarcinomas (78.8%) (p=0.007). The 5-year survival rate also differed significantly according to carcinoembryonic antigen level (69.3% in < 3 ng/mL, 23.8% in > 3 ng/mL; p < 0.001).
Conclusion
In pediatric patients, adenocarcinomas arise from various organs and are often diagnosed at advanced stages. Large, prospective studies for their accurate clinical characteristics and prognostic factors are needed.

Keyword

Adenocarcinoma; Pediatrics; Rare tumor

Figure

• 

  Fig. 1. (A) Survival distribution of all 80 patients. The 5-year overall survival rate was 57.9%. (B) Survival distribution according to the diagnosis. (C) Survival distribution according to the resection type. (D) Survival distribution according to the stage. The number of survival periods represents the year for each figure.

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  Fig. 2. Comparison of overall survival between the groups with gastrointestinal cancer (GI) and without GI (non-GI). The number of survival periods represents the year. GI, gastrointestinal cancers (gastric cancer, colorectal cancer, small bowel cancer, pancreatic cancer, cholangiocarcinoma); Non-GI, all cancers except for the abovementioned cancers.

• 

  Fig. 3. Comparison of survival in patients with carcinoembryonic antigen (CEA) level < 3 ng/mL and > 3 ng/mL. The value for the survival period represents the year.

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