Clinical features and outcome of solid pseudopapillary neoplasm: differences between adults and children.

OBJECTIVES To delineate the clinical and pathological characteristics of solid pseudopapillary neoplasm (SPN), compare them between adults and children, and determine the predictive features suggesting malignant potential. DESIGN Retrospective analysis of patients who underwent surgery for a pathologically confirmed SPN. SETTING Tertiary care referral center. PATIENTS Sixty-two consecutive patients who underwent surgery for a pathologically confirmed SPN between 1985 and 2006. MAIN OUTCOME MEASURES Demographic information and clinical presentation, radiological details, surgical data, pathological characteristics, postoperative course, and long-term survival. RESULTS Among 62 patients, 47 patients were adults (mean age, 36 years; range, 18-63 years) and 15 patients were children (mean age, 12 years; range, 8-13 years). A palpable mass was the most common presenting symptom in children (9 of 15; 60%) and an incidentally detected pancreatic mass, in adults (18 of 47; 38.3%) (P = .001). The mean tumor size in children was significantly larger than in adults (8.0 vs 6.0 cm; P < .03). In children, the tumor was located in the head of the pancreas (10 of 15; 66.7%) and in adults, in the body or tail (38 of 47; 80.9%) (P = .001). Nine patients (14.5%) had malignant SPN. There was no significant clinical factor suggesting malignant potential. Two patients had a tumor recurrence. They were still alive after debulking surgery. There were no tumor-related deaths. CONCLUSION Solid pseudopapillary neoplasm had different clinical features in adults and children. Because long-term survival can be achieved, even with the synchronous or metachronous metastatic lesions, SPN should be treated aggressively, with complete resection, even if this requires metastatectomy.