Der solid pseudopapilläre Pankreastumor (SPT) – eine seltene Raumforderung der Bauchspeicheldrüse

Abstract

SPT is a tumour of low malignancy. Radical resection is recommended for long-term recurrence-free survival. Chemotherapy may prolong survival in SPC with unresectable metastases.

Solid pseudopapillärer Tumor des Pankreas bei einem 12jährigen Mädchen – 7 Jahre Follow-up und histopathologische Reevaluation

“…In our patient the tumor caused more or less acute occurring and in fact persisting abdominal pain, which is observed in most cases of Frantz tumor [2,11,19,24,27,29]. In some cases the entity is discovered, e.g., after minor injuries [20], or in connection with palpable abdominal masses [2,7,14,18,20,23,29], loss of weight, further on nausea, vomiting [13,18,24] to hematemesis [2,13] in extremely rare cases. There are also cases in which patients do not show symptoms at all, so that the tumor is then found incidentally [6,14].…”

“…Solid-pseudopapillary tumors of pancreas are rare tumors that predominantly occur in young females and most frequently in their mid twenties [3,6,7]. Cases of older ( >70 years) or very young patients are reported rarely [2,17,25].…”

“…Solid-pseudopapillary tumor of the pancreas (so-called Frantz tumor) is a rare neoplasm with a frequency of about 0.17-2.7% of all pancreatic tumors [2,4,6,7,14,15,17,25]; only Yaacobi et al observe a higher incidence (7.3%) in their patient collective [27]. The tumor may occur at every age, but is predominantly found in young females (male to female ratio 1:10 to 1:20) [1,3,17,18,20,23].…”

“…Metastases are observed in about 15% of these cases [1,3]. The entity was given several names until 1996, when it was defined by the World Health Organization (WHO) as ''solid pseudopapillary tumor of the pancreas'' [7,17,25,28].…”

“…Only a few immunuhistochemical marker expressions are considered to be typical [1,5]. The origins of this tumor are still unknown, but it has been postulated to arise from primitive pancreatic cells (e.g., acinar cells, ductal epithelium, or endocrine cells) [5,20], pluripotent stem cells [13], or of cell lines of the female genital bud [7,23].…”