Solid-pseudopapillary tumor of the pancreas in a 12-year-old girl - 7 years follow-up and histopathological reevaluation: Case report and subject review

BACKGROUND: Solid-pseudopapillary tumor of the pancreas (so-called Frantz tumor) is a rare neoplasm of typically benign behavior, predominantly occurring in young females, but very rare in girls. METHODS: The clinical course of a 12-year-old girl suffering from such a tumor is presented and diagnostic and therapeutic methods are pointed out. Furthermore the relevance of the histological tumor classification respectively of recent immunohistochemical investigations with consideration of the actual literature will be discussed. The mentioned girl was admitted to our department of pediatric surgery because of upper abdominal pain, persistent for 12 hours. Initial examinations (physical examination, routine laboratory parameters, and abdominal ultrasound) showed a well-defined, spheric tumor (8 cm in diameter) with solid and cystic areas, without hints for metastases. The combination of the two facts - young girl and solid-cystic lesion of the pancreas - made us think about a Frantz tumor. By performing CT and MRT of the abdomen this diagnosis was hardened. In fact the patient underwent a Kausch-Whipple operation (modified by Traverso-Longmire) to perform a radical tumor removal. RESULTS: The clinical and radiological diagnosis was confirmed by histological examination of the tumor, i.e., the lesion was valued as solid-pseudo papillary neoplasm of the pancreas. Neither short-term or long-term surgical complications, nor metastases have been recorded in the follow-up (7 years up to now). An immunohistochemical reevaluation of the tumor material was carried out on occasion of the recent follow-up (April 2009); new aspects concerning tumor genesis could not be realized. CONCLUSIONS: Solid-pseudo papillary tumors of the pancreas are rare tumors. The majority of these tumors show benign behavior; very few malignant clinical courses have been reported. Despite rare occurrence of the tumor clinical presumption diagnosis possible, based on the fact that these tumors particularly affect young females and radiological findings show a tumor with solid and cystic parts. Radical tumor resection is the therapeutic method of choice. Because of the risk of injuring of the tumor an enucleation is discussed controversial. Despite special immunohistochemical and genetic examinations (influences of estrogenic hormones?) the origin of the tumor remains unknown.