I. I. Maizlin1, R. T. Russell1, M. B. Dellinger2, A. B. Goldin2, M. Goldfarb3, J. J. Doski4, A. Gosain5, M. Langer6, M. V. Raval7, J. G. Nuchtern8, S. A. Vasudevan8, K. W. Gow2, E. A. Beierle1 1University Of Alabama at Birmingham,Pediatric Surgery,Birmingham, Alabama, USA 2Seattle Children’s Hospital,Pediatric Surgery,Seattle, WA, USA 3John Wayne Cancer Institute At Providence St. John’s Health Center,Surgery,Santa Monica, CA, USA 4Methodist Children’s Hospital Of South Texas, University Of Texas Health Science Center-San Antonio,Surgery,San Antonio, TX, USA 5University Of Tennessee Health Science Center,Pediatric Surgery,Memphis, TN, USA 6Maine Children’s Cancer Program, Tufts University,Portland, ME, USA 7Emory University School Of Medicine, Children’s Healthcare Of Atlanta,Pediatric Surgery,Atlanta, GA, USA 8Baylor College Of Medicine, Texas Children’s Hospital,Pediatric Surgery,Houston, TX, USA
Introduction: While pancreatic islet cell tumors (ICTs) are rare pancreatic neoplasms in any age group, less than 10% of them occur in adolescents. While recent studies investigated the most appropriate treatment of pancreatic ICTs, as well as the effect of age on consequent survival, none of the patients examined were in the adolescent and young adult (AYA) category, with most patients in their 6th or 7th decade of life. The question arises, therefore, whether AYA patients would demonstrate a similar improvement in overall survival following ICT diagnosis. Therefore, we utilized a national database to describe the histological and clinical pattern of ICTs in AYA patients, comparing them to their older adult counterparts. Based on the limited studies on older populations, we hypothesized that AYA patients would have better overall survival following ICTs diagnosis.
Methods: A search of all AYA patients (15-39 yo) in the National Cancer Data Base (1998-2012) with ICT diagnosis was performed. Demographics, tumor characteristics, treatment modalities, and outcomes were abstracted and compared to adults (≥40 yo).
Results: After 11 patients were excluded for lack of survival data, 383 patients (56% female) were identified, with a median age of 27 (IQR 16-34) years at diagnosis. Non-Hispanic Caucasians comprised 65% of patients, 14% were Hispanic Caucasians, 14% African Americans, and 7% other ethnicities/races. Islet cell carcinoma was the most common histology (74%, n=285), followed by insulinomas (9%, n=36), gastrinomas (7%, n=28), mixed-cell tumors (6%, n=22), and VIPomas (3%, n=12). 24% of patients presented with early stage disease (Stage I:16%, Stage II:8%); 25% had advanced disease (Stage III:5%, Stage IV:20%). 267 patients underwent surgical resection, consisting of local excision 44%, Whipple procedure 37.5%, and total pancreatectomy 19%. Chemotherapy was utilized in 29% of cases and radiotherapy in 8%. With mean follow-up of 4.7 years, mortality was 36%. Cox regression analysis (n=361) demonstrated no difference in survival between different histologies (p=0.779). Comparison of AYAs to older adults demonstrated similar stage distribution (p=0.054), rates of comorbidities (p=0.727), and utilization of adjuvant therapies (chemotherapy p=0.153, radiation therapy p=0.666), with more extensive resections (p=0.001) in AYA patients as well as lower mortality rates (p<0.001).
Conclusions: This study found that AYA patients with ICTs had similar histologic distributions and comparable utilization of adjuvant therapies to older adult counterparts, but underwent more extensive resections and demonstrated a higher overall survival rate. While requiring further investigation, these results demonstrate a differentiation that may serve for evidence-based risk stratification of prognosis in ICT patients.