Pancreatic neoplasms are very rare in children. There are few large pediatric series in the literature, so multicentric studies are needed in order to add knowledge to the topic. The Latin American Pediatric Surgical Oncology Collaborative Group was recently created and chose to address this topic.
Retrospective review of clinical and surgical features of pediatric patients with pancreatic tumors cases diagnosed at 7 Latin American hospitals, between 2006 and 2018. Participating centers were invited by local pediatric surgical societies, and local ethical were approved
Forty-nine patients were included, 59% were female. The median age at operation was152 months. Most patients presented with abdominal pain and abdominal mass. Two had associated syndromes, one with Wolf-Parkinson and another with congenital hydrocephaly. Magnetic resonance imaging and computed tomographic scan were used in 87% and 38%, respectively. Four patients were metastatic (2 pancreatoblastoma with liver metastasis, 1 neuroendocrine tumor with lymph node metastasis and 1 desmoplasic tumor with liver metastasis), two patients had a metastatic tumor at the pancreas (Ewing’s and synovial sarcoma). Thirty-two patients underwent surgery (Whipple procedure in 16, distal pancreatectomy in 8 cases, tumorectomy in 7). The most common histology was solid pseudopapillary neoplasm (SPN) (n = 26), followed by pancreatoblastoma (n= 5), neuroendocrine tumor (n = 4), lymphoma (n = 3) and others (myofibrobastic tumor, desmoplasic tumor, insulinoma, indiferencied carcinoma, Ewing’s sarcoma, synovial sarcoma). Thirty-eight patients (84.4 %) are currently alive and disease free at a median follow-up of months. All SPN patients are alive. Five patients died: 2 pancreatoblastoma, 1 neuroendocrine tumor,1 lymphoma, and 1 sarcoma.
We report the first multicentric Latin American collaboration on pediatric surgical oncology, focused on pancreatic tumors. Patients with SPN have an excellent outcome, suggesting that limited surgical resection may be appropriate for these patients. Other malignant pancreatic tumors are more aggressive.