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.