A Rare Case of Solid Pseupapillary Tumor of the Pancreas in a Young Girl with Celiac Disease

Abstract

Solid papillary cystic tumors of the pancreas are rare entities and occur more frequently in women aged 20–40. We report a case of a 11-year old girl followed for celiac disease (CD) with a secondary diagnosis of a pseudocystic pancreatic tumor. This young girl was diagnosed with CD at the age of 9 after complaints of abdominal discomfort in the previous two months. Her sibling had been diagnosed with CD a few years earlier. Both her immunological work up and histological specimens were consistent with CD. In the following year her immunological tests progressively normalized, but her weight did not improve and her spine had to be evaluated for severe scoliosis. During work up an abdominal mass was noted. Subsequent evaluations identified a solid papillary cyst tumor of the pancreatic head. She was then operated on and the mass was fully enucleated. Histological findings confirmed the diagnosis and showed that the margins were free of disease. The latest abdominal ultrasounds have showed regular findings apart from two residual portal lymphatic nodes. She is now receiving pancreatic enzyme supplementations on the regular basis. This is the first case of a rare solid papillary tumor of the pancreatic head occurring in a young girl who also carries the diagnosis of CD.