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

Abstract

Background Solid pseudopapillary pancreatic tumors (SPPT) are an extremely rare entity in pediatric patients. Even if the role of radical surgical resection as primary treatment is well established, data about follow‐up after pancreatic resection in children are scant. Methods A retrospective review of data from the Italian Pediatric Rare Tumor Registry (TREP) was performed. Short‐term (<30 days) and long‐term complications of different surgical resections, as well as long‐term follow‐up were evaluated. Results From January 2000 to present, 43 patients (male:female = 8:35) were enrolled. The median age at diagnosis was 13.2 years (range, 7–18). Nine children had an incidental diagnosis, whereas 26 complained of abdominal pain and 4 of palpable mass. Tumors arose either from the head of pancreas (n = 14) or from body/tail (n = 29): only one patient presented with metastatic disease. Resection was complete in all patients (cephalic duodenopancreatectomy vs distal resection). At follow‐up (median, 8.4 years; range, 0–17 years), one recurrence occurred in a patient with intraoperative rupture. All patients are alive. Three pancreatic fistulas occurred in the body/tail group, whereas four complications occurred in the head group (one ileal ischemia, two stenosis of the pancreatic duct, and one chylous fistula). Conclusion Surgery is the best therapeutic option for these tumors; hence, complete resection is mandatory. Extensive resections, including cephalic duodenopancreatectomy, are safe when performed in specialized centers. Long‐term follow‐up should be aimed to detect tumor recurrence and to evaluate residual pancreatic function.

Laparoscopic Distal Pancreatectomy of a Solid Pseudopapillary Tumor (SPT) Achieves Long-Term Oncologic Safety and Multiorgan Preservation

“…8 9 Crocoli and coworkers have recently reported one of the largest series including detailed information of 43 pediatric patients with SPT. 10 The authors have found the typical female preponderance and the median age at diagnosis was 13.2 years.…”

“…13 Regarding the most prevalent localization of the tumor in the pancreas, there are contradictory findings in the literature. While some reports have found that the body/tail was most often affected, 10 others describe the pancreatic head as the most common localization 11 or an even distribution between head and tail. 8 In our patient, the mass arose from the tail of the pancreas in close relation to the spleen, left kidney, and adrenal gland.…”

“…11 In contrast, 21% of the children described in another study, by Crocoli et al, had an incidental diagnosis and the remaining patients were diagnosed on abdominal ultrasound performed due to abdominal pain or had a palpable mass. 10 A rare case with hemoperitoneum due to traumatic tumor rupture has also been described in the literature. 12 Taken together, prompt abdominal ultrasound in pediatric patients with unspecific abdominal pain seems to be a prerequisite for rapid diagnosis.…”

“…22 Crocoli et al described a series of 43 children with one case of tumor rupture (spillage). 10 However, it is unclear whether this patient was treated by open surgery or MIS.…”

Pancreatic Tumors

“…SPTs are considered to be low-grade malignancies with very good prognosis (Crocoli et al 2019;Ozcan et al 2018;Jaksic et al 1992;Shorter et al 2002;Perez et al 2009). Histologically, these tumors are extremely difficult to characterize.…”