One in a Million;

A CASE OF MUCINOUS RECTAL

CARCINOMA IN A PEDIATRIC PATIENT
Dennis Ragasa, MD
Resident in Training, Department of Surgery,
Mariano Marcos Memorial Hospital and Medical Center

BACKGROUND
Colorectal cancer is the third leading cause of cancer in adults, next to breast and lung cancers in women, and next to prostate and
lung cancers in men. It has a favorable treatment response and surgical resectability especially when diagnosed early made possible by
screening tools. However, the lack of screening in pediatric population leads to late diagnosis and poor prognosis. Mucinous carcinoma is
the most common histopathologic type seen in pediatric colorectal cancer located more common in the right colon.
CASE
This is the case of a 16-year old male presenting with more than invasion. Eight out of 16 (8/16) lymphnodes were positive for
a month history of vague to sharp anal pain more prominent after tumor cells including the distal resection margin. The proximal
defecation with associated occasional hematochezia, decreased resection margin was negative for tumor cells. Findings are
caliber of stool, significant weight loss (about 8% from previous 55 consistent with Stage IIIc (T3N2bM0) Mucinous Adenocarcinoma
to 51 kg), and anorexia. There was no heredofamilial history of of the Rectum. The perioperative period was uneventful and
disease nor cancer of any site. He was a non-smoker, non-alcoholic patient was discharged after 8 post-operative days.
beverage drinker, with no history of illicit drug use nor exposure to
radiation. Physical examination was unremarkable except for
fungating circumferential rectal mass on digital rectal examination.
Contrast-enhanced CT scan of the abdomen showed a
circumferential anorectal mucosal thickening. Biopsy of the
anorectal mass showed clusters of tumor cells floating in a sea of
mucin consistent with mucinous carcinoma.
A B
Abdominoperineal resection with creation of permanent
FIGURE 1
colostomy was executed on a supine-lithotomy position. Gross
examination of the abdomen showed no liver nodule, with Figure 1. CT scan plates showing
multiple internal iliac lymphnode adenopathy, and anterior circumferential mass with mucosal
adherence and invasion of the tumor to the posterior lobe of the thickening
prostate. Histopathology result of the resected organs revealed
large lakes of extracellular mucin mixed with collections of tumor
C
cells consistent with mucinous carcinoma of the rectum invading
up to the circumferential margin with no definite lymphovascular

A
Figure 2. The excised
part of the sigmoid, the
rectum, and the anus.
A— left anterolateral
view. B— right
posterolateral view.

FIGURE 2
B Figure 3. Mesocolon of
the excised sigmoid.

FIGURE 3

FIGURE 4

A B C D

Figure 4. Anorectal mass histopathologic examination revealing clusters of tumor cells floating in a sea of mucin. (A) specimen from an orectal mass biopsy.
(B, C, D) specimens from abdominoperineal resection which includes anorectal mass and Lymph nodes.

DISCUSSION
Colorectal Cancer (CRC) is is extremely rare in pediatric age
group with prevalence of 1.3-2 cases per million of population.
Mucinous adenocarcinoma is considered poorly differentiated
characterized by abundant mucous secretion comprising at least
50% of the tumor, with multiple gene mutations associated.
Mucinous Carcinoma is more commonly found in patients with
colon cancer than in those with rectal cancer (15% versus 9% of
patients, respectively), associated with an inferior response to
treatment, more frequently found in female patients,
predominantly located in the proximal colon, less common in
patients in Asian countries than the west, and more commonly
diagnosed in patients with inflammatory bowel diseases and in
patients with a history of pelvic or abdominal radiotherapy.
However, mucinous carcinoma is more common histological
presentation of colorectal cancer in pediatric population and there
is no associated risk factors in most pediatric cases.
CONCLUSION
Colorectal malignancy should no longer be excluded from consideration only on the basis of a patient’s age. There is a need of a high
index of suspicion and level of awareness for screening and early detection for patients presenting with complex symptomatology or
diagnostic dilemma.
REFERENCES
(1) Takalkar, UV et al. Carcinoma of colon in an adolescent: a case report with review of
literature. International Journal of Scientific Reports, [S.l.], v. 1, n. 2, p. 151-153, jun. 2015.
ISSN 2454-2164. Available at: <http://www.sci-rep.com/index.php/scirep/article/
view/47>. Date accessed: 22 Oct. 2017. doi:http://dx.doi.org/10.18203/issn.2454-
2156.IntJSciRep20150228.
(2) Mucinous colorectal cancer; advances in care European Society for Medical Oncology, 2016
(3) Antonio Muccillo, Edson Marchiori, Cláudia Renata Penna, et al., “Colon Mucinous
Adenocarcinoma in Childhood: A Case Report with Emphasis on Image Findings,” Case
Reports in Medicine, vol. 2010, Article ID 327634, 3 pages, 2010.
doi:10.1155/2010/327634
(4)Hugen N, Brown G, Glynne Jones R, et al. Advances in the care of patients with mucinous
colorectal cancer. Nature Reviews Clinical Oncology 2015; Published online 1 September.
doi:10.1038/nrclinonc.2015.140 (http://www.ncbi.nlm.nih.gov/pubmed/26323388)
Colorectal cancer usually presents with nonspecific symptoms
and vague clinical profile. Clinical features are similar to adults
with chronic abdominal pain, altered bowel habits, weight loss,
rectal bleeding, abdominal palpable mass, and anemia which is
associated with multiple differentials hence index of suspicion is
very low. Moreover, there is no established screening tools for
colorectal cancer in the pediatric population. This leads to
advanced disease at the time of diagnosis compounded by
undifferentiated histologic type and propensity to widespread
metastasis causing treatment challenges.
Early diagnosis with radical surgery and medical management is
cornerstone to improve outcome of the patients.
Abdominoperineal resection (APR) remained for many years the
main treatment option for most patients with low rectal cancer,
despite high local recurrence and poor prognosis.
(5) Redkar R G, Kulkarni B K, Naik A, Borwankar S S. Colloid carcinoma of rectum in a 11 year old
child. J Postgrad Med [serial online] 1993 [cited 2017 Oct 20];39:218. Available from: http://
www.jpgmonline.com/text.asp? 1993/39/4/218/600
(6) Colon and Rectum Cancer Staging. American Joint Committee on Cancer, 7th Edition. 2009
(7) Liu P, Bao H, Zhang X, et al. Better operative outcomes achieved with the prone jackknife vs.
lithotomy position during abdominoperineal resection in patients with low rectal cancer.
World J Surg Oncol. 2015 Feb 12;13:39. doi: 10.1186/s12957-015-0453-5.
(8) Park S, Hur H, Min BS, Kim NK. Short-term Outcomes of an Extralevator Abdominoperineal
Resection in the Prone Position Compared With a Conventional Abdominoperineal Resection
for Advanced Low Rectal Cancer: The Early Experience at a Single Institution.Ann Coloproctol.
2016 Feb; 32(1):12-9. doi: 10.3393/ac.2016.32.1.12. Epub 2016 Feb 29.
(9) Simha, V., Kapoor, R., Gupta, R., Bahl, A., & Nada, R. (2014). Mucinous adenocarcinoma of the
rectum: a poor candidate for neo-adjuvant chemoradiation? Journal of Gastrointestinal
Oncology, 5(4), 276–279. http://doi.org/10.3978/j.issn.2078-6891.2014.020