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Solid-Pseudopapillary Tumor of the Pancreas: a Population Based Study of 100 Patients
Attila Dubecz*1, Norbert Solymosi2, Michael Schweigert3, Hubert J. Stein1
1Surgery, Klinikum Nurnberg, Nuremberg, Germany; 2Complex Physics, Eötvös Lóránt University, Budapest, Hungary; 3Department of Thoracic Surgery, Klinikum Dresden Friedrichstadt, Dresden, Germany
BACKGROUND
Solid pseudopapillary tumor (SPT) of the pancreas (Frantz-Tumor) is a rare entity with only small surgical case-series reported in the literature. Natural history and results of non-operative management are unknown. In this study, we analyzed patients diagnosed with SPT in a population based sample.
METHODS
Using the National Cancer Institute's Surveillance Epidemiology and End Results Database (2004-2011), all patients with histologically diagnosed malignant SPT were identified. Epidemiologic and clinico-pathologic characteristics were assessed, long-term disease-related survival was calculated and compared between groups of patients undergoing surgery vs non-operative management.
RESULTS
There were 100 patients, with a mean age of 36.8 years. Most patients were female (64%) and of white race (64%). More than half (52%) of the tumors were in the tail of the pancreas. Average tumor size was 6.8 cm (range: 1.5-24 cm). Twenty percent of all tumors were >10 cm of size. Only 16% of the population did not undergo surgical treatment. Median tumor size was smaller (50mm vs. 55mm) in the non-operative group. There was a non-significant trend to younger age at diagnosis (36.3y vs. 39.9y; p= 0.4), to a higher percentage of whites (69% vs. 50%; p=0.16) and females (68% vs. 50%; p=0.7) in the surgery group. Median follow-up was 43.5 months. Overall disease related survival was significantly higher in patients undergoing surgical resection (97% vs. 62%). Most fatalities in the non-operative group (5 of 7) occurred within one year of the diagnosis. Prevalence of LN metastases in patients undergoing pancreatic resection was 4.7 % (4/84). In these patients, positive lymph node status was associated with worse overall five-year survival (N0: 71% vs N1: 5%).
CONCLUSION
Solid pseudopapillary tumors of the pancreas are rare, large tumors occurring predominantly in young white females. Surgical resection is associated with an excellent long-term survival for most patients. Large percentage of patients who do not receive operative treatment die within one year of diagnosis. Racial and gender disparities in access to surgical care might exist.
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