A177 AN UNUSUAL CULPRIT OF AN UPPER GASTROINTESTINAL BLEED IN A PAEDIATRIC PATIENT

Abstract

Abstract Background Leiomyomas are benign tumors originating from mesenchymal cells in the muscularis propria that present equally among the sexes usually as localized lesions after the fifth decade. Though rare, esophageal leiomyomas (EL) make up the majority of benign esophageal tumors and are typically located distally. Of all known cases, 2.6% are paediatric. In this population they are 1.71 times more common in females and are often diffuse. Most are slow growing and asymptomatic but when >5cm patients describe dysphagia, vomiting, pain, cough and anorexia. Gastric leiomyomas causing upper gastrointestinal bleeding (UGIB) in adults are reported. Aims To discuss a rare cause of UGIB in a child. Methods Case report. Results We present a 16-year-old Asian boy with no significant past medical history who was transferred to our institution for an UGIB following 3 days of dark black stools, 1 day of coffee ground emesis and pre-syncope. There was no history of non-steroidal anti-inflammatory use. At presentation he was in shock and received fluid resuscitation and a pantoprazole infusion. His hemoglobin dropped to 81 from 150g/L and corrected with 2 units of packed red blood cells. He was pale, non-dysmorphic with no cutaneous lesions, abdomen was non-distended and non-tender with no masses or hepatosplenomegaly. An abdominal ultrasound showed mild bowel wall thickening in the cecum and terminal ileum. Emergent upper endoscopy identified an ulcerated sessile mass at the gastro-esophageal junction. Biopsies revealed polypoid inflamed gastric mucosa with a focal intestinal metaplasia, negative for malignancy. Magnetic resonance scan characterized a 3.8x4.3x2.5cm lesion in the stomach cardia not extending beyond the serosa with suggested causes including EL and gastrointestinal stromal tumor (GIST). Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) demonstrated a 2.8x1.8cm well circumscribed homogenous hypoechoic mass in the muscularis propria of the distal esophagus. Histopathology showed bland tumor cells with eosinophilic cytoplasm and nuclei that did not demonstrate atypia. Immunohistochemistry (IHC) stains were positive for smooth muscle actin and desmin. CD117, S-100 and DOG1 were negative. Findings were therefore in keeping with a leiomyoma and not a GIST. He was referred to the Cancer Genetics clinic for consideration of hereditary leiomyomatosis renal cell cancer and General Surgery for resection. Conclusions This case highlights a rare cause of UGIB in a child. EL are scarcely found in children and at present there are no reports of EL causing UGIB. They can be associated with Alport Syndrome or Familial Leiomyomatosis. IHC is an essential diagnostic tool, enabling differentiation of EL from GIST which is important as the latter has malignant potential. EL can be monitored with endoscopy if small but are surgically resected if symptomatic, ulcerated, or growing. Funding Agencies None