A rare cause of intestinal obstruction in children: signet-ring cell adenocarcinoma of the colon

Abstract

Background: Signet-ring cell adenocarcinoma of the colon is well-recognized in adult patients who are extremely rare and not well-documented in children. Our study aims to raise awareness about this rare disease and its long-term outcomes.

Methods: We retrospectively evaluated patients with signet-ring cell colon adenocarcinoma.

Results: Six patients, three boys and three girls, with a mean age of 14.83 (range, 13-17 years), presented with signs of intesti-nal obstruction and were diagnosed with signet-ring cell colon adenocarcinoma. All patients had air-fluid levels on abdominal X-ray. Abdominal ultrasonography of all patients revealed subileus. Abdominal computed tomography was performed in five patients, and pre-operative colonoscopy was conducted in two patients before the emergency intervention. All of the patients underwent emergent exploratory laparotomy with the preliminary diagnosis of acute abdomen. In two patients, debulking surgery followed by a stoma was performed. The remaining four patients were treated with anastomosis following intestinal resection. All girls had metastases on the ovary. One of the patients died due to the burden of multiple metastases in the early period, and three died in the sixth post-operative year. We have been following the remaining two patients since then.

Conclusion: Although signet-ring cell carcinomas (SRCCs) are rare, they should be considered in the differential diagnosis of acute abdomen and intestinal obstruction in pediatric patients. Despite early diagnosis and treatment, SRCC has a poor prognosis in the pediatric population.

Figure 1

Air-fluid levels seen in the right lower quadrant of the abdomen X-ray in Case 3.

Figure 2

Histopathologic images of the tumors in Case 3 (a-c) and Case 4 (d-f). (a) Signet-ring cells (yellow arrow) adjacent to the crypts (white arrow) of the colon mucosa. (b) Signet-ring cells (arrow) dispersed in the mucinous stroma. (c) Lymph node metastasis of the tumor. Signet-ring cells (yellow arrow) adjacent the lymphocytes (white arrow) of the lymph node parenchyma. (d) Peritoneal surface invasion of the tumor. Signet-ring cells adjacent to the black ink (arrows). (e) Signet-ring cells dispersed in the mucinous stroma. (f) Ovarian metastasis. Signet-ring cells (arrows) scattered within the ovarian stroma.

Figure 3

MRI showing a mass in the left ovary in Case 3.

Figure 4

A CT image consistent with a mass lesion in the transverse and descending colon in Case 4.

Figure 5

A colonic mass of approximately 10 cm in length that started at the level of the splenic flexure and involved the descending colon, completely occluding the lumen and protruding beyond the lumen, in Case 4.