Mucinous Adenocarcinoma of the Rectum in a 13-Year-Old Child - A Case Report

Mostafa Zain1, Mostafa Kotb1, Basmal Abdulkareem1, Yasmine Elwany2, Dina Abdallah1, Hanan Tayel1

1 Alexandria Faculty of Medicine, Alexandria, Egypt;
2 Alexandria Medical Research Institute, Alexandria, Egypt.

Conflict-of-interest statement: The author(s) declare(s) that there is no conflict of interest regarding the publication of this paper.

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Correspondence to: Mostafa Kotb, Alexandria Faculty of Medicine, Alexandria, Egypt.
Email: rashad@alexmed.edu.eg
Telephone: +201062333868

Received: January 3, 2020
Revised: February 22, 2020
Accepted: February 27, 2020
Published online: June 21, 2020


Although colorectal carcinoma (CRC) is the second most common primary gastrointestinal system malignancies in children after liver tumors, it is extremely rare in pediatric age. Due to the lack of awareness of the disease, diagnosis is usually delayed till it is in advanced stage, leading to a poor prognosis as compared to adults. We present a case of CRC in a 13-year-old girl who presented with abdominal pain, vomiting and unintentional weight loss. Clinical examination and imaging did not reveal a clear pathology. On exploration, a marked circumferential thickening of the wall of the sigmoid colon and the rectum was noted. Biopsies proved to be a mucinous adenocarcinoma of the rectosigmoid, infiltrating the wall till subserosal fat.

Key words: Colorectal cancer; Mucinous adenocarcinoma; Signet-ring cells

© 2020 The Authors. Published by ACT Publishing Group Ltd. All rights reserved.

Zain M, Kotb M, Abdulkareem B, Elwany Y, Abdallah D, Tayel H. Mucinous Adenocarcinoma of the Rectum in a 13-Year-Old Child - A Case Report. Journal of Gastroenterology and Hepatology Research 2020; 9(3): 3228-3230 Available from: URL: http://www.ghrnet.org/index.php/joghr/article/view/2771


Colorectal carcinoma (CRC) comprises around 1% of pediatric neoplasms, and after primary liver tumors, it is the second most common primary gastrointestinal system malignancies in children[1]. Due to the lack of awareness of the disease, diagnosis is usually delayed till it is in advanced stage, leading to a poor prognosis as compared to adults[2]. We report a case of mucinous adenocarcinoma of the rectum in a 13-year-old boy without any risk factors. We also review the clinical and pathological characteristics of such a rare entity.


A 13-year-old boy presented to our department with fever, bilious vomiting and abdominal pain since 6 days. There was a history of weight loss (about 25 kg) and loss of appetite for the last 3 months. On examination, there was generalized tenderness and rigidity all over the abdomen. Computed tomography (CT) of the abdomen and pelvis showed mild supra and infracolic pneumoperitoneum as well as moderate to marked partially loculated fluid collection all over the abdomen.

On entry to the abdomen, about 1 liter of pus was aspirated. Moreover, a marked circumferential thickening of the wall of the sigmoid colon and the rectum was noted (Figure 1). This lesion was extending down to about 2 cm above the anal verge. Resection of this mass was not feasible due to severe inflammation and friability of the surrounding tissues as well as its distance from the anal verge. Therefore, two full thickness biopsies were taken from the sigmoid and subsequently the procedure was terminated.

Multiple greyish brown tissue fragments were submitted to the pathology department, they were totally processed. Microscopic examination of H&E stained sections revealed an infiltrative neoplastic growth infiltrating through the whole wall thickness: mucosa, submucosa and musculosa. The tumor is composed of mucin secreting signet ring cells forming nests and acini and were floating within mucin lakes. The cells showed pleomorphism, hyperchromatism, with marked nuclear atypia. Foci of necrosis were noted as well (Figure 2).

Figure 1 Intraoperative photo showing a marked circumferential thickening of the wall of the sigmoid colon and the rectum.

Figure 2 Histologic features of the tumor. A: H&E stained section showing infiltration of mucosa and submucosa by tumor masses (H&Ex100). B: H&E stained section showing splayed musculosa by tumor nests (H&Ex100). C: A section showing acini and clusters of signet ring cells within mucin lakes (H&Ex100). D: High power image showing signet ring cells (H&Ex400).


CRC is uncommon in children, with only few cases had been reported in literature[3-9]. The reported peak age among the reported case is 15 years old, whilst the youngest reported case was a 9-month-old female infant[5].

Vomiting and abdominal pain are among the most common symptoms in pediatric CRC patients[19]. Other possible symptoms include weight loss, change in bowel habits and anemia depending on the location of the tumor[10]. Unfortunately, these symptoms are nonspecific in children, and they may mimic many common functional gastrointestinal disorders [11]. The duration of symptoms before diagnosis is usually long, ranging from 2 to 6 months (median of 3 months)[10].

The disease usually occurs sporadically[12]; nevertheless, 10% to 30% of CRCs in children have predisposing factors for instance, familial adenomatous polyposis (FAP), Crohn’s disease, ulcerative colitis and Peutz-Jegher syndrome. One of the most important genetic mutations in pediatrics CRC is high microsatellite instability (MSI) that results from a DNA mismatch repair system. It is considered the hallmark of the early-onset CRC, Lynch syndrome, which is also known as hereditary nonpolyposis colorectal cancer syndrome (HNPCC)[5].

Regrettably, due to the vague nature of its presentation, children usually present with an advanced stage of the disease at initial diagnosis and a higher incidence of more aggressive tumors as well[5,9]. Whilst adults are usually affected with moderately differentiated adenocarcinoma, mucinous adenocarcinoma predominates in the pediatric age group despite being rare in adults[26]. The mucin is known to absorb water, swell and invade the nearby tissues; thus, promoting spread of malignant cells. The tumors has the capability to attain a huge size as the result of the pooling mucin as well as the interference made by mucopolysaccharide coating to the immune recognition of carcinoma cells[14]. The signet ring subtype is notorious for its worst prognosis owing to its very rapid growth, regional lymph node involvement and diffuse peritoneal seeding at the initial presentation[14].

Surgery offers the only curative modality for localized CRCs. Nevertheless, patients with stage ≥ III should undergo adjuvant chemotherapy to eradicate micrometastases in order to reduce disease recurrence and increase the cure rate[15]. Compared to adults, metastasis during initial presentation is considerably higher in pediatrics compared to adults (37% vs 18%)[16]. The most common sites of distant metastasis are liver, lung, lymph nodes, and peritoneum[16]. Indicators of poor prognosis are high tumor staging, incomplete excision, mucinous histology, signet-ring cells proportion > 10% and absence of in-situ component[13]. Despite the advances in diagnosis and treatments, the overall 5-year survival is around 75% in adult patients but, unfortunately, only 51% in pediatric population[4].

To sum up, should there is a significant unintentional loss of weight along with change in bowel habits, a search for underlying malignancy, even in the absence of a positive family history or predisposing cancer syndromes, should be made.


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