Recurrence of Solid Pseudopapillary Neoplasms of the Pancreas: Results of a Nationwide Study of Risk Factors and Treatment Modalities

Abstract

Background Solid pseudopapillary neoplasms of the pancreas (SPPN) can relapse very late, but little is known about risk factors for recurrence and optimal treatment. We aimed to identify risk factors for recurrence and to analyze treatment modalities in all French pediatric cases of SPPN over the past 20 years. Material and methods Data were collected from pediatric oncologists and surgeons, and also from adult pancreatic surgeons in order to identify late recurrences. Results Fifty‐one patients (41 girls) were identified. Median age at diagnosis was 13.1 years [8.7–17.9]. Abdominal pain was the commonest presenting symptom (32/49, 65%). The tumor was located in the pancreatic head in 24 patients (47%). Preoperative biopsy or cytology was performed in 14 cases (28%). All patients were operated with a median of 23 days [0–163] after diagnosis. The rate of postoperative morbidity was 29%. With a median follow‐up of 65 months [0.3–221], the overall and event‐free survival was 100% and 71%, respectively. Seven patients (13.7%) relapsed with a median of 43 months [33–94] after initial surgery. Six were treated surgically, either alone (n = 3) or with perioperative chemotherapy (n = 2) or hyperthermic intraperitoneal chemotherapy (n = 1). One patient in whom further treatment was not feasible was still alive at last news. Risk factors for recurrence were positive surgical margins (P = 0.03) and age less than 13.5 years at diagnosis (P = 0.03). Conclusions SPPN recurrence in this pediatric series was a rare and late event that did not undermine overall survival. Complete surgical removal of recurrent tumors appears to be the best option.

Solid pseudopapillary and malignant pancreatic tumors in childhood: A systematic review and evidence quality assessment

“…After screening titles and abstracts, 50 studies were retrieved for full‐text evaluation. Ultimately, 32 papers reporting on a total of 489 pediatric patients with pancreatic tumors satisfied predetermined search criteria and were included in this systematic review (Figure ) . Nine studies were conducted in the United States, five in Korea, four in Canada, and the rest of them came from Europe and Japan.…”

“…Data on postoperative complications was available in 20 papers . Pancreatic fistulae were reported in 11% of SPTs, ileus was described in 25% of PBLs, and fluid collections were found in 14.3% of exocrine pancreatic tumors (Table ).…”

“…A total of 35 patients were treated with a classical Whipple procedure . Mean patient age was 11.7 (±7.2) years and the female:male ratio was 1.6:1.…”

Outcome after surgery for solid pseudopapillary pancreatic tumors in children: Report from the TREP project—Italian Rare Tumors Study Group

“…Local recurrence, as well as metastatic disease, is often related to peripancreatic tissue infiltration, neural/vascular invasion, or lymph node spread configuring an aggressive behavior. Moreover, abdominal blunt injury could be a cause of tumor spread . In this respect, the impact of tumor spillage is still controversial, explaining some degree of reluctance to perform preoperative biopsies.…”

“…However, malignant behavior is rare and always associated with specific histological features, such as vascular or neural invasion, peripancreatic infiltration, and nodal metastases. In this respect, complete surgical resection is the mainstay of treatment to achieve excellent long‐term outcomes . Local recurrence and metastases are rare and often reported after incomplete resection, as well as expression of intrinsic malignancy of the tumor .…”