Introduction
Pancreaticoduodenectomy (PD), infrequently performed in children due to the rarity of pancreatic tumors in this age group, can lead to significant morbidity in 30 to 40 % of patients, including exocrine and endocrine pancreatic deficiency, dumping syndrome, delayed gastric emptying, diarrhea, growth retardation, pancreatic leaks and strictures1,2. Pancreatoblastoma and solid pseudopapillary tumors (SPN) are the most frequent pancreatic tumors in the first and second decades of life, respectively2; numerous other malignant tumors have been reported, however.1–5
Rhabdomyosarcoma (RMS), the most common soft tissue sarcoma in children and adolescents, rarely presents primarily in the gastrointestinal tract. RMS has been described involving the biliary tract, the liver or the duodenum1,3,6–11. Two cases of a primary pancreatic RMS have been reported, one of pleomorphic histology and one of unspecified subtype12,13.
This article describes the first case of fusion-positive RMS arising in the pancreas of an adolescent patient, details the severe side effects she had to overcome after PD and during treatments, and explores the role of biopsy for pancreatic tumors in pediatrics.