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Lipoblastoma-Related Volvulus in a Nine-Year-Old Female.

Luke Gingell¹, Aryana Sharrak², Kristen Hawes²

¹Medicine, Michigan State University College of Human Medicine, Grand Rapids, USA.

|July 16, 2024

Summary

This case study highlights a rare intraabdominal lipoblastoma in a child that caused a small bowel obstruction. Early detection and surgical intervention are crucial for managing this pediatric tumor, with long-term monitoring advised.

Keywords:

acute volvulus lipoblastoma pediatric hematology-oncology plag-1 upper gastrointestinal surgery

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Area of Science:

Pediatric Surgery
Abdominal Imaging
Surgical Pathology

Background:

Lipoblastomas are rare, benign pediatric neoplasms originating from embryonal adipocytes.
Typically presenting as superficial masses, intraabdominal lipoblastomas with complications are exceptionally uncommon.

Observation:

A 9-year-old female presented with abdominal pain, initially found to have a large omental mass.
A follow-up visit revealed signs of small bowel obstruction due to mass migration and volvulus.
CT imaging demonstrated a hypodense omental mass with mesenteric 'swirl sign' and bowel dilation.

Findings:

The patient underwent emergency surgery for small bowel obstruction caused by a lipoblastoma.
Resection included 20 cm of necrotic small bowel due to volvulus.
Cytogenetics confirmed lipoblastoma, associated with PLAG1 gene rearrangements.

Implications:

This case underscores the potential for rare intraabdominal lipoblastomas to cause life-threatening complications like volvulus and obstruction.
Prompt surgical management and long-term surveillance with imaging (ultrasound or MRI) are essential for pediatric patients with lipoblastoma.
Understanding the genetic underpinnings (PLAG1) aids in diagnosis and prognosis.