Malignant pancreatic tumor other than solid pseudopapillary tumor in pediatric patients

Abstract

Pancreatic tumors, except solid pseudopapillary tumors (SPTs), are rare in pediatric patients. Herein, we report various types of pancreatic tumors in pediatric patients and review the literature regarding their treatments and prognosis. We retrospectively reviewed the data of pediatric patients who underwent surgery for pancreatic tumors, excluding SPTs, between January 2009 and December 2019 at Seoul National University Children's Hospital. A total of 35 pediatric patients were identified as having undergone surgery for pancreatic tumors. Of these patients, 30 were excluded because the tumor was identified as an SPT. The diagnoses of the five remaining (non-SPT) pancreatic tumors were pancreatic neuroendocrine tumor, mixed acinar neuroendocrine carcinoma, kaposiform hemangioendothelioma, and intraductal papillary mucinous neoplasm. All five patients survived; however, recurrence and liver metastasis were observed in one patient. The detailed demographics, treatments, and prognosis of each patient were reviewed. Despite the rarity and low i ncidence of pancreatic tumors in pediatric patients, four types of non-SPT tumors are reported here. Hence, the possibility of these should not be overlooked, especially since the diagnosis and adjuvant treatment differ vastly between the tumor types.

Case Report: Pancreatic and hepatic kaposiform hemangioendothelioma presenting as consumptive coagulopathy and right hepatic atrophy

“…We reviewed the literature and retrieved eight published cases of pancreatic KHE with sufficient information ( Table 1 ) ( 11 – 18 ). Almost all cases occurred in in the pancreatic head and had KMP; presenting with biliary obstruction symptoms, such as obstructive jaundice and biliary vomiting.…”

Management of pancreatic kaposiform hemangioendothelioma with sirolimus in a pediatric patient: a case report and literature review

“…In animal models, the anti-angiogenic and anti-lymphangiogenic effects of mTOR in vascular tumors including KHE have been supported (22,23), and this preclinical testing provides the evidence for clinical application of sirolimus. Following clinical applications over a number of years (1,(9)(10)(11)14), sirolimus has recently been recommended as a new treatment for KHE with or without KMP. In a study of sirolimus with more than 2-year follow-up, 73% (19/26) of patients showed shrinkage with single sirolimus treatment, while the remaining cases received sirolimus combined with vincristine and steroids and showed shrunken tumors (24).…”

“…Pancreatic tumors are relatively rare in children, especially so in neonates and infants ( 10 ). However, pancreatic KHE often preferentially affects infants, as shown in our series of reported cases, where all pediatric cases were younger than 2 years old ( Table 1 ).…”