Papillary cystic tumor of the pancreas, so-called Frantz tumor, is a very rare tumor in children. Only 157 cases had been reported since 1959. The clinical manifestations of this disease are usually a slowly growing abdominal mass with or without abdominal pain. It occurs predominantly in young females, and its pathogenesis is still unknown. Surgical resection is usually curative, and its prognosis is excellent. Four adolescent girls with acute or chronic abdominal pain were found to have papillary cystic tumor of the pancreas at our hospital in the past 4 years. They all have a variable clinical presentation. Papillary cystic tumor of the pancreas should therefore be considered one of the differential diagnoses of abdominal pain or abdominal mass in adolescent girls.
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