ARTICLE

Colorectal Carcinoma in Children and Adolescents - Case Reports and Literature Review

A B S T R A C T

Colorectal carcinoma (CRC) is extremely rare among pediatrics, particularly before puberty. It usually begins with nonspecific signs and symptoms, so the index of suspicion is low, consequently, the diagnosis may be delayed. Reports in the literature indicate that poor prognostic factors are more common in children than in adults, resulting in a worse outcome. Our objective is to report clinical profile, treatment and prognosis of CRC in children and adolescents admitted in our institution, and to perform a systematic review of reports in the bibliography.

Keywords

Colon carcinoma, child, adolescents

Introduction

Colorectal adenocarcinoma (CRC) is the third most common malignancy in the adult population, surpassed only by lung and breast cancers. However, only 1-4 % of all CRC occurs in patients younger than 25-30 years old, being extremely rare among children, particularly before puberty. However, it is the most common gastrointestinal primary malignancy after liver tumors according to the Surveillance, Epidemiology, and End Results (SEER) database [1, 2].

Initial signs and symptoms may be nonspecific. Available data showed that the most common symptom was abdominal pain (54%), change in bowel habits (38%), rectal bleeding (31%), weight loss (19.7%), nausea and vomiting (17%), and iron deficiency anemia (77%). In advanced disease there may be intestinal obstruction and perforation associated with a poor prognosis. The acute onset is more common in pediatrics (>20%) [1].

Because of the rarity of the pathology, these signs and symptoms are often underestimated or misunderstood as other more common childhood problems such as gastroenteritis, which may lead to a delay in diagnosis; the median time elapsing between the first symptoms and diagnosis was reportedly 3 months for patients younger than 20 years old, as opposed to 1 month for those over 20 [1, 3-4]. Furthermore, it has been hypothesized that the delay in diagnosis could partly explain the advanced stages in children [5]. CRC in children differs from adults mainly in higher incidence of aggressive histological features (poorly differentiated, signet-ring or mucinous adenocarcinoma subtypes), advanced stage, more microsatellite instability and worse survival rate [1].

Patients and Methods

We retrospectively analyzed all patients younger than 20 years old with diagnosis of CRC referred to Hospital de Pediatría Dr. J.P. Garrahan, Buenos Aires, Argentina between September 1987 and December 2020. A systematic review of the literature was performed using keywords and MeSH terms "Adolescent" AND/OR "Child" AND "Colorectal Neoplasms". Case reports were qualitatively assessed and analyzed according to predefined criteria: n >5 and younger than 30 years old.

Result / Case Reports

Fifteen patients younger than 20 years old (range 11-19) were admitted during the study period (Table 1). Ten were male and five were female. The median time of delay in diagnosis was 2.37 months (range 8 days-5 months). The most common symptoms were abdominal pain and weight loss; 5 patients reported acute surgical abdomen.

Two patients had been previously diagnosed and treated for a high-grade glioma, one of them with Neurofibromatosis type 1. Other two patients had increased risk factors for developing CRC: One with Crohn's disease and one with ulcerative colitis.

Unfavorable histotypes were more frequent: mucinous adenocarcinoma was observed in 9 patients, 5 being signet ring cell carcinoma. Poorly differentiated and undifferentiated tumors (grades III and IV) were seen in 6 of the 15 patients. Rectosigmoid was the most common primary site (8 patients). Fourteen patients reported an advanced stage of the disease (Dukes stage III-IV); more than half had distant metastases, seen in 9 of the 15 patients. At the moment of this analysis only 2 patients were alive, thirteen died because of the disease. The 1-year progression-free survival was 28,5%. Median mortality time was 9.8 months. An important limitation for our series is the lack of adequate immunohistochemical and genetic analyses on our patients.

Literature Review

Twenty-six full-text articles were retrieved (Table 2). The youngest reported patient was a 9-month-old girl [6]. Most reports confirm that pediatrics have unfavorable histotypes, advanced stages and double rates of metastases than adults, as we observed in our series. The 5 years overall survival reported for children is between 10-48% vs 60-81% for adults [2, 5, 7-9]. In our series only two patients were alive at the time of this study. The risk factors with statistical significance identified were: aggressive histological features, advanced Dukes stages, metastasis, lymph node involvement, complete surgical resection, use of adjuvant chemotherapy [1, 3, 10-11].

The median delay in diagnosis reported is between 1 and 4.5 months, coincident with our series [5, 10-15]. This worse prognosis than in adults may be due to the aggressive behaviour and/or the delayed diagnosis resulting in an advanced stage of the disease, with a lower chance of complete resection, the mainstay of its treatment [5, 23, 27, 30].

CRC in children and adolescents has a strong association with inherited cancer susceptibility syndromes [1, 7, 9, 17, 21, 29]. In our series, we report 2 cases with previous diagnosis of inflammatory bowel disease (1 Crohn's disease and 1 ulcerative colitis), and 2 patients with previous diagnosis of High-grade glioma, one of them with Neurofibromatosis type 1.

Conclusion

CRC poses many challenges to pediatric oncologists because of the low incidence, the delay in diagnosis may substantially contribute to the poorer prognosis, added to the higher prevalence of worse prognosis factors. Also, there is a strong association with hereditary cancer predisposition syndromes such Lynch Syndrome or Familial adenomatous polyposis, with manifestations that, even when mostly appear in adults, the adequate early screening allows its prevention and timely treatment. Added to that, it is important for the pediatrician and the pediatric oncologist to be able to provide genetic counseling for all the family.

Conflicts of Interest

None.

ARTICLE INFO

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Article Info

Article Type

Case Series and Review of the Literature

Publication history

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© 2023 Mathey Marina. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Hosting by Science Repository.

DOI: 10.31487/j.SCR.2022.09.01

AUTHOR INFO

Author Info

Mathey Marina

Pennella Carla

Zubizarreta Pedro

Corresponding Author

Mathey Marina
Department of Hematology and Oncology, Hospital de Pediatría S.A.M.I.C. “Prof. Dr. Juan P. Garrahan”, Buenos Aires, Argentina

FIGURES & TABLES

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