Rhabdomyosarcoma of the gallbladder

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric population, for 45% of soft tissue sarcomas in children. Commonly located in the head and neck, urogenital system, extremities and torso, RMS of the biliary system is rare. We present a specific case of RMS in the gallbladder of a 4-year-old girl who presented with epigastric abdominal pain and vomiting. Her work-up revealed transaminitis and tumefactive sludge in the gallbladder neck. After she had persistent symptoms and repeated work-up, the patient underwent a laparoscopic cholecystectomy and was found to have cholecystitis intraoperatively with cholelithiasis. However, pathology revealed botryoid embryonal RMS with positive microscopic margins. This case demonstrates the difficulty in diagnosing gallbladder RMS and the importance of considering malignancy in pediatric biliary disease.