Department of Pediatric Surgery, Istanbul University, Istanbul Medical Faculty, İstanbul-Türkiye.
Department of Pediatric Surgery, Sisli Hamidiye Etfal Training and Research Hospital, İstanbul-Türkiye.
Ulus Travma Acil Cerrahi Derg. 2023 Jul;29(7):798-805. doi: 10.14744/tjtes.2023.64257.
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.
We retrospectively evaluated patients with signet-ring cell colon adenocarcinoma.
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.
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.
结直肠印戒细胞腺癌在成年患者中较为常见,但在儿童中极为罕见,且相关文献记载较少。本研究旨在提高对这种罕见疾病及其长期预后的认识。
我们回顾性评估了患有印戒细胞结肠腺癌的患者。
6 名患者,3 男 3 女,平均年龄 14.83 岁(范围 13-17 岁),表现为肠梗阻症状,被诊断为印戒细胞结肠腺癌。所有患者的腹部 X 线均显示气液平面。所有患者的腹部超声均显示不完全性肠梗阻。5 名患者进行了腹部 CT 检查,2 名患者在紧急干预前进行了术前结肠镜检查。所有患者均因初步诊断为急腹症而行急诊剖腹探查术。其中 2 例患者行肿瘤减灭术+造口术,4 例患者行肠切除吻合术。所有女孩的卵巢均有转移。其中 1 例患者因早期多发转移导致病情过重而死亡,3 例患者在术后第 6 年死亡。此后我们一直对其余 2 例患者进行随访。
尽管印戒细胞癌(SRCCs)较为罕见,但在儿童急腹症和肠梗阻的鉴别诊断中应考虑到这一疾病。尽管早期诊断和治疗,但 SRCC 在儿科人群中的预后仍较差。