Gastric signet‐ring cell carcinoma in a child: A clinical case study

Abstract

Gastric signet‐ring cell carcinoma (SRCC) is a rare form of gastric cancer characterized by mucus‐rich tumor cells forming a distinctive ring‐like appearance and is exceedingly rare in children, accounting for less than 1% of all childhood malignancies. We report a case of a 14‐year‐old patient who presented with melena, hematemesis, and syncope. Upper gastrointestinal endoscopy revealed an ulcerative mass located in the gastric cardia. Histopathological examination confirmed gastric adenocarcinoma demonstrating signet‐ring cell features. The patient underwent laparoscopic‐assisted radical proximal gastrectomy, esophagogastric anastomosis, abdominal drainage, and a D2 lymphadenectomy, which includes dissection of both perigastric and second‐tier lymph nodes. The final diagnosis was SRCC of the stomach. Postoperative Tumor Node Metastasis (TNM) staging was stage III (T3N0M0). Follow‐up indicates that the patient remains in good health and has remained symptom‐free for 5 years. Given the rarity of this malignancy and the limited number of pediatric case studies, there is an urgent need to accumulate additional clinical and immunohistochemical data to enhance understanding and improve diagnostic accuracy.

1. INTRODUCTION

Gastric signet‐ring cell carcinoma (SRCC) is a rare and aggressive subtype of adenocarcinoma characterized by intracellular mucin displacing the nucleus, resulting in the characteristic “signet‐ring” appearance of the cells. ¹ It often presents with nonspecific symptoms and is associated with a worse prognosis than other histological types of gastric cancer, frequently leading to late‐stage diagnosis. ² Herein, we report an exceedingly rare case of SRCC in a pediatric patient. This report aims to share our clinical experience alongside a literature review, to enhance understanding of this rare malignancy and contribute to the available clinical data for treatment and long‐term follow‐up.

2. CASE REPORT

A 14‐year‐old Chinese male presented to the emergency department following a 2‐h episode of melena, hematemesis, and syncope. His medical history was notable for testicular torsion at the age of 13, which had been managed with emergent surgical orchiopexy. On admission, his vital signs were stable, including a blood pressure of 110/55 mmHg, heart rate of 74 beats per minute, respiratory rate of 20 breaths per minute, and a normal body temperature of 36.7°C. Physical examination revealed abdominal tenderness at the xiphoid and left lower quadrant, along with pallor of the bilateral conjunctivae. No other significant abnormalities were noted.

Laboratory testing revealed a white blood cell (WBC) count of 10.7 × 10⁹/L (reference: 4.5–11.0 × 10⁹/L), hemoglobin level of 103 g/L (reference: 130–175 g/L), and hematocrit of 29% (reference: 40%–50%). Renal function was within normal limits, except for a mildly elevated blood urea nitrogen (BUN) of 12 mmol/L (reference: 2.5–7.1 mmol/L) and the presence of mild proteinuria. Coagulation studies showed a prothrombin time of 14.5 s (reference: 11–13.5 s), and lactate was elevated at 2.54 mmol/L (reference: 0.5–2.2 mmol/L). Electrocardiography demonstrated normal sinus rhythm.

Computed tomography (CT) imaging showed no significant abnormalities. However, upper gastrointestinal angiography revealed irregular mucosal patterns in the gastric fundus, raising suspicion for a lesion.

The following day, an upper gastrointestinal endoscopy was performed (Figure 1), which revealed mucosal lacerations and ulceration in the gastric cardia. Biopsies were obtained, and hemostasis was achieved using titanium clips. Three days later, histopathological analysis confirmed the presence of SRCC of the gastric cardia and fundus (Figure S1). A follow‐up esophagogastroduodenoscopy (EGD) performed 2 days postbiopsy (Figure 2) demonstrated retained titanium clips at the cardia and fundus, accompanied by mucosal edema and erythema. The gastric wall architecture was partially obscured by postprocedural inflammatory changes. Surgical intervention was postponed due to the patient's anemia and general weakness. Following supportive care, the patient's condition improved, and he was deemed fit for surgery.

Figure 1.

Gastroscope photos: (A, B) Endoscopic appearance at initial presentation. No obvious abnormalities were observed in the duodenal bulb and descending part; (C, E) the gastric fundus showed extensive mucosal congestion, edema, and erosion (red arrows). A cardia ulcer was visible along the lesser curvature, and no other ulcers or masses were seen elsewhere; (D, F) the stomach body opened and closed well, with a clear dentate line. A 0.5 × 0.6 cm ulcer (red asterisk) was visible at the lesser curvature, with a white coating on the surface and a soft texture. (A) Duodenal bulb; (B) descending duodenum; (C) gastric fundus; (D) stomach body; (E) gastric fundus; (F) cardia.

Figure 2.

Gastroscope photos: Follow‐up endoscopy 5 days posthemostasis. In the gastric fundus, an ulceration change approximately 0.7 × 0.8 cm in size is visible at the 12 o'clock direction from the cardia (red arrow). Surrounding the ulcer, there is mild irregular elevation with a hard texture, poor elasticity, and tendency to bleed easily. I‐scan staining can reveal the disappearance of microvessels and the disorganization of the surface structure at the lesion site. Indigo carmine staining shows a clear boundary of the lesion. Multiple congestive erosions with a soft texture are visible at the gastric fundus fornix. (A) Cardia; (B–H) gastric fundus.

The patient subsequently underwent laparoscopic‐assisted subtotal gastrectomy with esophagogastric anastomosis and D2 lymphadenectomy. The tumor, measuring approximately 1 × 1.5 cm, was located on the lesser curvature of the gastric fundus, just distal to the cardia. There was no evidence of serosal invasion or adjacent organ involvement. Regional lymphadenopathy was addressed through excision of enlarged nodes. The greater omentum and gastrocolic ligament were divided, and lymph nodes at stations No. 4sb, 4d, 4sa, and 10 were resected. After a 6‐cm gastric resection, an esophagogastric anastomosis was constructed using a circular stapler.

Postoperatively, the patient was maintained nil per os and received total parenteral nutrition. On postoperative Day 7, he was transitioned to a clear liquid diet, and by Day 11, to a semiliquid diet (Figures S2 and S3). He tolerated enteral nutrition well, experienced only minimal weight loss, and regained baseline weight within 3 months. Pathological examination confirmed tumor invasion into the muscularis propria with negative surgical margins. Immunohistochemistry revealed weak C‐erbB‐2 expression, negative p53 staining, and positive staining for EGFR, VEGF, CA19‐9, CDX2, Topoisomerase II, and ERCC1.

During 5 years of follow‐up, the patient exhibited no signs of tumor recurrence or metastasis. He continued to undergo annual endoscopic surveillance and tumor marker monitoring. A minor finding during the first month of follow‐up included mild esophagitis at the anastomotic site.

3. DISCUSSION

SRCC accounts for approximately 8%–30% of all gastric cancers and is associated with a poor prognosis due to its aggressive nature and propensity for late‐stage detection. ³ Despite an overall decline in gastric cancer incidence—largely attributed to widespread Helicobacter pylori eradication—SRCC cases have demonstrated an increasing trend. ⁴ In the present case, the patient presented with upper gastrointestinal bleeding, which led to the diagnosis of SRCC in the gastric cardia. Although the tumor was small in size, its challenging anatomical location complicated endoscopic visualization and diagnosis.

Pediatric SRCC is exceedingly rare, with fewer than 50 cases reported in children worldwide. ⁵ Most pediatric patients are diagnosed at advanced stages (stage III or IV), with a notable predilection for the gastric cardia. ³ Prognosis is generally poor in both pediatric and adult populations, with 5‐year survival rates ranging from 20% to 40%, depending on disease stage at diagnosis and the success of surgical resection. ⁴ Pediatric cases may present with more aggressive histopathological characteristics and a higher frequency of peritoneal dissemination.

Radical surgical resection—either subtotal or total gastrectomy with D2 lymphadenectomy—remains the mainstay of curative treatment for localized disease across all age groups. ⁶ While adjuvant chemotherapy is recommended for advanced stages, pediatric‐specific treatment guidelines remain underdeveloped due to the extreme rarity of cases. ⁷ The biological behavior of SRCC, including its tendencies for lymphovascular invasion and peritoneal metastasis, appears consistent across age groups; however, pediatric tumors may demonstrate particularly aggressive histological features. ⁸

In this case, successful tumor resection and absence of recurrence over a 5‐year follow‐up period likely reflect the benefits of early detection, timely surgical intervention, and comprehensive postoperative care. Although pediatric SRCC is rare, this case underscores the importance of maintaining a high index of suspicion for gastrointestinal symptoms in children and suggests that early intervention can improve outcomes even in aggressive malignancies.

In summary, this rare case of pediatric SRCC diagnosed at a resectable stage emphasizes the importance of early suspicion and timely intervention. Multidisciplinary management is essential to optimize outcomes in pediatric patients with rare gastrointestinal malignancies. ⁸

4. CONCLUSION

Pediatric SRCC is exceptionally rare, and this case underscores the importance of recognizing early signs of gastric malignancy in children. Early diagnosis and timely intervention, as demonstrated here, can result in favorable clinical outcomes. Multidisciplinary care and sustained long‐term follow‐up are essential for the effective management of such rare and aggressive malignancies. Further research is urgently needed to develop evidence‐based treatment protocols and enhance clinical outcomes in pediatric SRCC.

CONFLICT OF INTEREST STATEMENT

The authors declare no conflicts of interest.

ETHICS STATEMENT

This study adhered to the ethical principles outlined in the Declaration of Helsinki (2013 revision) and complied with the guidelines of the International Committee of Medical Journal Editors (ICMJE). All procedures involving human participants were reviewed and approved by the Institutional Review Board (IRB) of The fifth affiliated hospital of Zun yi Medical University (Zhuhai hospital). Written informed consent was obtained from the patient's legal guardian for publication of this case report and accompanying images. The consent document explicitly acknowledges the following: (1) de‐identification of all personal health information, (2) potential educational/research use of the material, and (3) waiver of future claims related to data dissemination.

Supporting information

Supplemental Figure S1. pathological findings: (4a and 4b) Biopsies were taken from the cardiac and fundic parts of the stomach with tissue pieces measuring 0.05, 0.1, 0.2, and 0.3 centimeters in size. The pathological results revealed the presence of mucinous lakes with a large number of signet ring cells (indicated by red arrows) in the mucosa of the cardia and fundus, accompanied by diffuse infiltration of inflammatory cells (indicated by blue arrows), suggesting a diagnosis of signet ring cell carcinoma. (4c and 4d) The pathological findings indicate that the tumor invaded and ruptured the intrinsic muscle layers of the stomach and esophagus (indicated by black arrows) and locally infiltrated the superficial muscle layer of the esophagus (indicated by green arrows).

Supplemental Figure S2. Gastroscopic photos: Congestion, edema, and erosion were observed at the esophageal stump and gastric anastomosis site (yellow arrow). The esophageal lesser curvature side at the anastomosis showed orange mucosa crawling with a smooth surface. There was food residue in the residual stomach, and at the 11 o'clock direction of the residual stomach, a broad‐based polypoid elevation (red arrow) was visible, with a reddened surface, measuring approximately 0.4*0.5 centimeters in size, and a soft texture; the residual gastric and intestinal anastomosis site was congested and edematous, with slight swelling at the 5 o'clock position (green arrow), with a smooth surface and soft texture. No ulcers or masses were seen within 20 centimeters of the enterotomy limb.

Supplemental Figure S3. pathological findings: Hyperplasia of the fibrous tissue in the lamina propria of the mucosa (red asterisk), accompanied by infiltration of lymphocytes (yellow arrows) and plasma cells (indicated by the green arrows); a small number of lymphocytes (yellow arrows) and plasma cells (green arrows) were observed in the lamina propria of the mucosa, showing changes of chronic inflammation (at the intestinal stump and gastric anastomosis site, and in the residual stomach).

Wang J, Yang Y, Wei J, et al. Gastric signet‐ring cell carcinoma in a child: a clinical case study. JPGN Rep. 2026;7:285‐288. 10.1002/jpr3.70084