[The solid pseudopapillary tumor (SPT)--a rare neoplasm of the pancreas].
BACKGROUND
In general, the rare SPT is a tumour of low malignancy predominantly affecting young women. The outcome after radical resection is favourable. In exceptional cases the tumour presents as solid pseudopapillary carcinoma (SPC) with typical malignant features and even metastases. Unresectable liver metastases can be treated with RFA, TACE or chemotherapy.


METHODS
We retrospectively reviewed the surgical approach, immunohistochemistry and clinical outcome in five female patients (1998--2007).


RESULTS
The mean age was 16 years (range: 13-47 years). For radical tumour removal a pancreato-duodenectomy (n = 3), a distal pancreatectomy (n = 1) and an enucleation (n = 1) were performed. We encountered a mean tumour diameter of 8 cm (range: 6-15 cm), an angioinvasion (3/5) and a lymphatic infiltration (1/5). After 30 to 101 months follow-up four patients were free of recurrence. Chemotherapy has resulted in a survival of over 98 months in a case of SPC with liver metastases.


CONCLUSIONS
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.
Foltys
D
D
Abteilung für Transplantationschirurgie, Chirurgie von Leber, Gallenwegen und Pankreas, Klinikum der Johannes-Gutenberg-Universität Mainz, Mainz. foltys@transplantation.klinik.uni-mainz.de
Moench
C
C
Burck
I
I
Hoppe-Lotichius
M
M
Schad
A
A
Teufel
A
A
Heise
M
M
Otto
G
G
D000293:Adolescent; D000328:Adult; D002291:Carcinoma, Papillary / Q000175:diagnosis* / Q000601:surgery*; D005260:Female; D006801:Humans; D008875:Middle Aged; D010180:Pancreatectomy; D010190:Pancreatic Neoplasms / Q000175:diagnosis* / Q000601:surgery*; D016896:Treatment Outcome