Malignant Pancreatic Tumors in Children and Young Adults: Evaluation of 228 Patients Identified through the Surveillance, Epidemiology, and End Result (SEER) Database

Abstract

Background:

Malignant pancreatic tumors are rare in young patients, few epidemiologic data are available. We reviewed prognostic factors and outcome of 228 patients <30 years with malignant pancreatic tumors identified through the U.S. National Cancer Institute’s SEER (Surveillance, Epidemiology, and End Results) Public-use Database from 1973 to 2004.

Methods:

Cases were grouped using the ICD-O-3. 5-year overall survival (OAS) was assessed by gender, ethnicity, SEER stage, and 5-year age intervals using univariate and Cox regression analysis.

Results:

228 patients with malignant pancreatic tumors were identified, resulting in an incidence of 0.46/million (100 carcinomas, 85 endocrine tumors, 8 solid pseudopapillary neoplasms (SPN), 11 pancreatoblastomas) in the USA. OAS was worse in males than females (37% vs. 55%, p=0.005). OAS according to stage was 87%, 68%, 21% for local (n=54), regional (n=42), distant metastatic disease (n=108), respectively. OAS of patients with carcinoma was 33%, endocrine tumors 58%, SPNs 88%, pancreatoblastomas 66%. Cox regression revealed stage (p=< 0.001), histology (p=< 0.001), age group (p=0.05) to be independent prognostic factors.

Conclusion:

Malignant pancreatic tumors are extremely rare in children and young adults. Entities change over the age groups towards more carcinomas with worse outcome in older patients. Tumor stage, histology and age group are important predictors for outcome. International collaboration is needed to learn more about pediatric pancreatic tumors.

Zusammenfassung

Hintergrund:

Maligne Pankreastumoren sind im Kindes- und jungen Erwachsenenalter selten, epidemiologische Daten stehen nur begrenzt zur Verfügung. Wir untersuchten die Charakteristika und prognostischen Parameter von 228 Patien­ten <30 Jahren mit malignem Pankreastumor, die in der öffentlich zugänglichen SEER- (Surveillance, Epidemiology, and End Results) Datenbank des US-amerikanischen National Cancer Institute von 1973 bis 2004 registriert wurden.

Methoden:

Die Fälle wurden entsprechend der ICD-O-3 gruppiert. Das 5-Jahres-Gesamtüberleben (OAS) wurde unter Verwendung univariater Analyse und des Cox-Regressionsmodells nach Geschlecht, ethnischer Gruppe, SEER-Tumorstadium und Altersgruppe ermittelt.

Ergebnisse:

228 Patienten mit malignem Pankreastumor wurden identifiziert, entsprechend einer Inzidenz von 0,46/Mio (100 Karzinome, 85 endokrine Tumoren, 8 solide pseudopapilläre Neoplasien (SPN), 11 Pankreatoblastome) in den USA. Männliche Patienten zeigten ein geringeres OAS als weibliche Patienten (37% vs. 55%, p=0,005). OAS nach Stadium war 87% für Patienten mit lokalisiertem Tumor (n=54), 68% für regionale Metastasierung (n=42), 21% für Fernmetastasen (n=108). OAS der Patienten mit Karzinom war 33%, endokrinem Tumor 58%, SPN 88%, Pankreatoblastom 66%. Die multivariate Analyse identifizierte Stadium (p=< 0,001), Histologie (p=< 0,001) und Altersgruppe (p=0,05) als unabhängige prognostische Faktoren.

Schlussfolgerung:

Maligne Pankreastumoren sind sehr selten bei Kindern und jungen Erwachsenen. Die Inzidenzen der Entitäten verändern sich über die Altersgruppen hin zu Karzinomen mit geringerer Überlebensrate bei jungen Erwachsenen. Tumorstadium, Histologie und Alter haben Einfluss auf das Überleben. Für weiterführende klinische Analysen sind internationale Kooperationen notwendig.

Key words

primary pancreatic tumors - pediatric malignant tumors - epidemiology - SEER database - rare tumors - young adults

Schlüsselwörter

Primäre Pankreastumoren - pädiatrische maligne Tumoren - Epidemiologie - SEER Datenbank - seltene Tumoren - junge Erwachsene

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