Pancreatic Adenocarcinoma in a Young Patient Population-12-Year Experience at Memorial Sloan Kettering Cancer Center

ArticleinJournal of Surgical Oncology 100(1):8-12 · July 2009with9 Reads
DOI: 10.1002/jso.21292 · Source: PubMed
Abstract
There is a dearth of data in a younger population of patients with pancreatic ductal adenocarcinoma (PAC) regarding epidemiology, genetics, prognosis, and outcome. This report examines a large cohort of patients with PAC <or=45 years of age evaluated at MSKCC over a 12-year period. A retrospective analysis of patients referred to MSKCC with PAC identified from the institutional tumor registry, who were <or=45 years on the date of the diagnostic biopsy, between January 1995 and February 2008, was performed. Information reviewed included demographics, clinical and pathological staging, surgical management, therapy, date of relapse, death or last follow-up. Survival curves were estimated using the Kaplan-Meier method and compared using the log-rank test. One hundred thirty-six cases of PAC, age <or=45 years at diagnosis, were identified. Seventy-four (54%) females, 62 (46%) males. Age range: 24-45; 4, 38, and 94 patients in age groups 20-29, 30-39, 40-45 years, respectively. Fifty (37%) had a smoking history. Fourteen (10.3%) had a positive family history of PAC. Thirty-five (25.7%) underwent a curative resection for localized disease. Twenty-eight (20.1%) presented with locally advanced, inoperable disease. Sixty-eight (50%) presented as AJCC Stage IV. Twenty-three (37%) of those resected underwent adjuvant chemoradiation. Thirteen received adjuvant gemcitabine. The median overall survival for the entire cohort was 12.3 months (95% CI 10.2-14.0 months). The median overall survival for the patients with locally resectable disease was 41.8 months (95% CI 20.3-47 months). The median overall survival for the patients who presented with locally advanced, unresectable disease was 15.3 months (95% CI 12-19.3 months). The median overall survival for those who presented with metastatic disease was 7.2 months (95% CI 5.2-9.5 months). This is the largest reported cohort of young patients with PAC <or=45 years of age. The data suggest that patients with stages I-II disease may have an improved prognosis, however the prognosis for stages III-IV patients appears to be similar to the typical (older) patient population with PAC.
    • "There was, however, no difference in the obesity ratio in this study as compared to the general population (9 vs. 12%). Genetic factors have been discussed as a plausible explanation for EOPC.In this study fewer patients with possible hereditary cancer were found compared to other reports [7,21]. In larger studies on ductal adenocarcinoma an incidence of hereditary pancreatic ductal adenocarcinoma are found in 5-10%. "
    [Show abstract] [Hide abstract] ABSTRACT: Pancreatic ductal adenocarcinoma (PDAC) represents the fourth cause of death in cancer with a 5-year survival rate of less than 1-2%. Early onset pancreatic cancer (EOPC), i.e. patients below 50 years of age, is infrequently described. The present study aimed to determine the epidemiology, demographic incidence and prognosis of EOPC and to identify the characteristics that might distinguish EOPC. 576 consecutive patients with PDAC diagnosed from January 1993 to December 2008 at the University Hospital of Lund, Sweden. 65 different parameters were compared with a historic cohort of pancreatic cancer patients as well as with matched controls. 33 patients (5.7%) with PDAC were 50 years or younger. The overall survival was 170 days compared to 240 days for matched controls (p=n.s.). Patients with EOPC were diagnosed at a significantly more advanced stage, i.e. distant metastasis, (52%) as compared to matched controls (30%; p=0.04). EOPC-patients received more treatment than the compared PDAC-cohort. EOPC constituted 5.7 % of all PDAC, presenting at an advanced stage with frequent metastases and poor survival. The role of genetic alterations and the potential influence of environmental factors for EOPC are to be further investigated.
    Full-text · Article · May 2011
    • "Our data were in concordance with those observations. Interestingly, none of 136 EOPC cases in the study of Duffy et al. [4] and none of EOPC cases in the present study were associated with inherited syndromes associated with elevated risk of developing PC. Only a single patient with EOPC in the present study was diagnosed with familial PC. "
    [Show abstract] [Hide abstract] ABSTRACT: Early onset pancreatic cancer (EOPC) constitutes less than 5% of all newly diagnosed cases of pancreatic cancer (PC). Although histopathological characteristics of EOPC have been described, no detailed reports on precursor lesions of EOPC are available. In the present study, we aimed to describe histopathological picture of extratumoral parenchyma in 23 cases of EOPCs (definition based on the threshold value of 45 years of age) with particular emphasis on two types of precursor lesions of PC: pancreatic intraepithelial neoplasia (PanIN) and intraductal papillary mucinous neoplasms (IPMNs). The types, grades, and densities of precursor lesions of PC were compared in patients with EOPCs, in young patients with neuroendocrine neoplasms (NENs), and in older (at the age of 46 or more) patients with PC. PanINs were found in 95.6% of cases of EOPCs. PanINs-3 were found in 39.1% of EOPC cases. Densities of all PanIN grades in EOPC cases were larger than in young patients with NENs. Density of PanINs-1A in EOPC cases was larger than in older patients with PC, but densities of PanINs of other grades were comparable. IPMN was found only in a single patient with EOPC but in 20% of older patients with PC. PanINs are the most prevalent precursor lesions of EOPC. IPMNs are rarely precursor lesions of EOPC. Relatively high density of low-grade PanINs-1 in extratumoral parenchyma of patients with EOPC may result from unknown multifocal genetic alterations in pancreatic tissue in patients with EOPCs.
    Full-text · Article · Mar 2011
  • [Show abstract] [Hide abstract] ABSTRACT: Acute, persistent abdominal pain due to ruptured pancreatic carcinoma and perforated stomach is extremely rare during pregnancy. We evaluated a woman at 34 weeks of gestation presenting with uterine contractions. Computed tomography scanning revealed a large retroperitoneal mass, and her blood carbohydrate antigen 19-9 level was elevated. Immediately after an emergency cesarean delivery, pancreatic cancer was detected, and pancreatoduodenectomy was performed. The patient underwent chemotherapy and remains disease-free at 2 years. Delayed diagnosis and treatment are associated with high morbidity of both neonate and mother in cases of pancreatic cancer during pregnancy. Computed tomography scanning and carbohydrate antigen 19-9 levels are useful for diagnosis, after which radical surgery should be performed immediately in late pregnancy.
    Article · Aug 2010
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