TY - JOUR
T1 - Primary malignant pancreatic neoplasms in children and adolescents
T2 - A 20 year experience
AU - Rojas, Yesenia
AU - Warneke, Carla L.
AU - Dhamne, Chetan A.
AU - Tsao, Kuojen
AU - Nuchtern, Jed G.
AU - Lally, Kevin P.
AU - Vasudevan, Sanjeev A.
AU - Hayes-Jordan, Andrea A.
AU - Cass, Darrell L.
AU - Herzog, Cynthia E.
AU - Hicks, M. John
AU - Kim, Eugene S.
AU - Austin, Mary T.
PY - 2012/12
Y1 - 2012/12
N2 - Background: Malignant pancreatic neoplasms in children and adolescents are rare. The clinical presentation, pathologic characteristics, management, and outcomes at two institutions are discussed. Methods: We retrospectively reviewed all pediatric patients (age < = 18 years) treated for malignant pancreatic neoplasms at two institutions between 1991 and 2011. Results: Thirty-one patients were identified with median age of 14.7 years (4-18 years). The most common histology was solid pseudopapillary tumor (SPT) (n = 22, 71%) followed by neuroendocrine tumors (n = 4, 13%), pancreatoblastoma (n = 4, 13%), and one unclassified spindle cell neoplasm (3%). Most patients presented with abdominal pain (n = 22, 71%). Complications included pancreatic leak, pseudocyst formation, pancreatitis, pancreatic insufficiency, and small bowel obstruction. The overall 1- and 5-year survival was 96% (95% CI 74%-99%) and 78% (95% CI 43%-93%). Median follow-up among patients alive at the end of follow-up was 20 months (< 1 month-16.2 years). Patients with SPT had better overall survival compared to patients with neuroendocrine tumors or pancreatoblastomas (Log-rank; p = 0.0143). Conclusion: The majority of pediatric and adolescent patients present with SPTs which are usually resectable and associated with an excellent prognosis. Other histologic subtypes more often present with distant metastases and portend a worse prognosis.
AB - Background: Malignant pancreatic neoplasms in children and adolescents are rare. The clinical presentation, pathologic characteristics, management, and outcomes at two institutions are discussed. Methods: We retrospectively reviewed all pediatric patients (age < = 18 years) treated for malignant pancreatic neoplasms at two institutions between 1991 and 2011. Results: Thirty-one patients were identified with median age of 14.7 years (4-18 years). The most common histology was solid pseudopapillary tumor (SPT) (n = 22, 71%) followed by neuroendocrine tumors (n = 4, 13%), pancreatoblastoma (n = 4, 13%), and one unclassified spindle cell neoplasm (3%). Most patients presented with abdominal pain (n = 22, 71%). Complications included pancreatic leak, pseudocyst formation, pancreatitis, pancreatic insufficiency, and small bowel obstruction. The overall 1- and 5-year survival was 96% (95% CI 74%-99%) and 78% (95% CI 43%-93%). Median follow-up among patients alive at the end of follow-up was 20 months (< 1 month-16.2 years). Patients with SPT had better overall survival compared to patients with neuroendocrine tumors or pancreatoblastomas (Log-rank; p = 0.0143). Conclusion: The majority of pediatric and adolescent patients present with SPTs which are usually resectable and associated with an excellent prognosis. Other histologic subtypes more often present with distant metastases and portend a worse prognosis.
KW - Children
KW - Outcome
KW - Pancreatic tumor
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U2 - 10.1016/j.jpedsurg.2012.09.005
DO - 10.1016/j.jpedsurg.2012.09.005
M3 - Article
C2 - 23217876
AN - SCOPUS:84870720477
SN - 0022-3468
VL - 47
SP - 2199
EP - 2204
JO - Journal of pediatric surgery
JF - Journal of pediatric surgery
IS - 12
ER -