Clinical and biological behavior of pancreatic solid pseudopapillary tumors: report on 31 consecutive patients.

Background: Solid pseudopapillary tumors (SPTs) represent one of the most uncommon histotypes of all exocrine pancreatic neoplasms.

Aim: To delineate the clinical presentation and biological behavior of SPT and evaluate the efficacy of treatment.

Methods: Retrospective analysis of 31 patients (27 female, 4 male, mean age of 34 years, (range 7-56)) who underwent surgical resection with a definitive histological diagnosis of SPT.

Results: Tumor detection was incidental in the 55% of cases. Symptoms were abdominal discomfort (n=10), jaundice (n=2), weight loss (n=6), vomiting (n=5), and a palpable abdominal mass (n=4). The neoplasm was localized in the pancreatic head in 10 patients and in the body-tail in 20 cases; the main diameter ranged from 2 to 20 cm (mean 5.4). At the radiological work-up, the neoplasm was solid in 87% of cases and delimited by a capsule in 39%. An internal necrotic-hemorrhagic area was present in 29% of cases. Calcifications were noticed in two patients and septa in one. None of the patients had metastases at the time of diagnosis. In 9 cases, pancreaticoduodenectomy was performed, while 15 patients underwent a left pancreatectomy (4 spleen preserving), 6 a middle pancreatectomy, and 1 enucleation. There was no postoperative mortality with an overall morbidity of 35%. At a median follow-up of 58.2 months (12-229 range), all patients are alive without evidence of local recurrence, metastasis, diabetes, or exocrine insufficiency.

Conclusions: Solid pseudopapillary tumor (SPT) is an indolent neoplasm with low-grade biological aggressiveness, making surgical treatment successful despite its large size.