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Multifocal adenocarcinoma of the stomach in a child with common variable immunodeficiency
M E Conley1, M M Ziegler, S Borden
1Department of Pediatrics, Children's Hospital of Philadelphia, PA 19104.
Insights
A rare case of stomach adenocarcinoma in an 11-year-old child with common variable immunodeficiency is presented. Despite treatment, the child succumbed to infections post-surgery, highlighting the challenges in managing such complex pediatric cases.
Area of Science:
- Pediatric Oncology
- Gastroenterology
- Immunology
Background:
- Describes a rare pediatric case of adenocarcinoma of the stomach.
- Highlights the association between common variable immunodeficiency (CVID) and gastrointestinal diseases.
- Discusses the clinical presentation of gastritis, achlorhydria, and panhypogammaglobulinemia in a young patient.
Observation:
- The patient experienced severe growth failure, recurrent infections, and abdominal pain starting at age six.
- Radiographic studies at age 11 revealed a fungating gastric mass, despite negative prior endoscopic biopsies.
- A radical gastrectomy was performed for multifocal adenocarcinoma.
Findings:
- The adenocarcinoma showed no invasion of the submucosa or local lymph nodes.
- The patient died six months post-gastrectomy due to Candida septicemia and pneumonia.
- Autopsy confirmed no evidence of tumor recurrence.
Implications:
- Underscores the complex interplay between immunodeficiency and malignancy in pediatric patients.
- Suggests the need for vigilant monitoring and early detection strategies in children with CVID and persistent GI symptoms.
- Emphasizes the critical role of managing underlying immunodeficiency to improve outcomes in associated malignancies.
Abstract:
The clinical course of a child who developed an adenocarcinoma of the stomach at 11 years of age is described. At 6 years of age, the child was evaluated for abdominal pain, weight loss, and vomiting. She was found to have hemorrhagic, atrophic gastritis, achlorhydria, and panhypogammaglobulinemia. The gastritis improved with corticosteroid therapy, but relapsed each time that the steroid dosage was tapered. The clinical course was marked by severe growth failure, recurrent infections, and intermittent abdominal pain. Radiographic studies done when the patient was 11 years of age demonstrated a large fungating mass on the lesser curvature of the stomach. Endoscopy and biopsies done 1 year previously had not revealed any sign of malignancy. A radical gastrectomy was performed. Microscopic studies revealed multifocal adenocarcinoma of the stomach with no evidence of invasion of the submucosa or local lymph nodes. The patient died of Candida septicemia and pneumonia 6 months after the gastrectomy. There was no evidence of recurrence of the tumor on autopsy. The relationship between common variable immunodeficiency and gastrointestinal disease is described.