Clinical Characteristics and Outcomes From an Institutional Series of Acinar Cell Carcinoma of the Pancreas and Related Tumors

2002 ◽  
Vol 20 (24) ◽  
pp. 4673-4678 ◽  
Author(s):  
Kyle D. Holen ◽  
David S. Klimstra ◽  
Amanda Hummer ◽  
Mithat Gonen ◽  
Kevin Conlon ◽  
...  
Keyword(s):  
Cell Carcinoma ◽  
Median Survival ◽  
Acinar Cell ◽  
Demographic Data ◽  
Univariate Analysis ◽  
Acinar Cell Carcinoma ◽  
Ductal Adenocarcinoma ◽  
Rank Test ◽  
High Recurrence Rate ◽  
Localized Disease

PURPOSE: Acinar cell carcinoma is a rare tumor of the exocrine pancreas. Clinical features such as prognostic information, survival, and treatment outcomes are unknown. We present the largest retrospective review to date. PATIENTS AND METHODS: Thirty-nine patients with pathologically confirmed acinar neoplasms of the pancreas were identified between August 1981 and January 2001. Demographic data, tumor characteristics, and treatment information were obtained by chart review. Survival probabilities were estimated by using the Kaplan-Meier method and compared using the log-rank test. RESULTS: The median survival for all patients was 19 months. On the basis of a univariate analysis, the patients’ stage of disease correlated significantly with survival. The median survival of patients with localized disease was 38 months, versus 14 months for those presenting with metastases (P = 0.03). Patients who could be treated with surgery as first-line therapy had a longer survival time (36 months) compared with those who did not have surgery (14 months). Two of 18 patients who received chemotherapy and three of eight patients who received radiation had a major response. CONCLUSION: The survival curves suggest a more aggressive cancer than pancreatic endocrine neoplasms but one that is less aggressive than ductal adenocarcinoma of the pancreas. Those patients who present with localized disease have a much better prognosis than those who present with metastases. There is a high recurrence rate after complete surgical resection, suggesting that micrometastases are present even in localized disease and that adjuvant therapies may be indicated. Chemotherapy and radiation afford disappointing results, however, and novel therapies are needed.

Pancreatic acinar cell carcinoma: A multi-center series on clinical characteristics and treatment outcomes.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e16253-e16253
Author(s):  
Vishwajith Sridharan ◽  
Mari Mino-Kenudson ◽  
James M. Cleary ◽  
Osama E. Rahma ◽  
Kimberly Perez ◽  
...  
Keyword(s):  
Cell Carcinoma ◽  
Treatment Outcomes ◽  
Acinar Cell ◽  
Metastatic Disease ◽  
Demographic Data ◽  
Case Series ◽  
Acinar Cell Carcinoma ◽  
Rank Test ◽  
Survival Difference ◽  
Localized Disease

e16253 Background: Acinar cell carcinoma (ACC) is a very rare tumor of the exocrine pancreas, representing less than 1% of all pancreatic malignancies. The majority of data regarding ACC are limited to small case series. Methods: This is a retrospective study conducted at a large healthcare system from 1996-2019. Patients with pathologically confirmed ACC were included, and demographic data, tumor characteristics, and treatment outcomes were abstracted by chart review. Survival curves were obtained by using the Kaplan-Meier method and compared using the log-rank test. Results: Sixty-six patients with ACC were identified. The median patient age at diagnosis was 64, and 42% presented with metastatic disease. The majority presented with abdominal pain or pancreatitis (69%), and laboratory parameters did not correlate with tumor size, metastatic disease, or survival. Several somatic abnormalities were noted in tumors (BRCA2, TP53, and mismatch-repair genes). In patients with localized disease that underwent resection, the median time to develop metastatic lesions was 13 months. The median overall survival (OS) was 24.7 months from diagnosis, with a survival difference based on metastatic disease at diagnosis (median 15 vs 38 mos). Surgery conferred OS benefit in non-metastatic cases (p = 0.006) but not metastatic cases (p = 0.22), and chemotherapy provided OS benefit in metastatic disease (p < 0.01). Patients with metastatic ACC treated after 2010 utilized more platinum-based agents, and there was a OS benefit to FOLFOX or FOLFIRINOX chemotherapy compared to gemcitabine or capecitabine-based regimens (p = 0.006). Conclusions: Pancreatic ACC patients often present with advanced disease. Surgery confers survival benefit among patients presenting with localized disease. The use of FOLFOX or FOLFIRINOX chemotherapy regimens was associated with improved OS in metastatic patients. These data add to our knowledge in this rare malignancy, and improves understanding about the genomic underpinnings, prognosis and treatment for acinar cancers.

scholarly journals The contemporary trend in worsening prognosis of pancreatic acinar cell carcinoma: A population-based study

PLoS ONE ◽  
2020 ◽  
Vol 15 (12) ◽  
pp. e0243164
Author(s):  
Nie Duorui ◽  
Bin Shi ◽  
Tao Zhang ◽  
Chuyao Chen ◽  
Chongkai Fang ◽  
...  
Keyword(s):  
Cell Carcinoma ◽  
Acinar Cell ◽  
Clinical Characteristics ◽  
Proportional Hazards ◽  
Survival Rates ◽  
Acinar Cell Carcinoma ◽  
Population Based ◽  
Pancreatic Acinar Cell ◽  
Cox Proportional Hazards ◽  
Rank Test

Background Primary acinar cell carcinoma (ACC) is a rare exocrine tumor of the pancreas with unclear clinical characteristics. Our goal was to determine the incidence and update the clinical characteristics and outcomes of ACC. Methods Through the Surveillance, Epidemiology, and End Results (SEER) database, we identified 252 patients with the latest diagnosis of ACC (2004–2016). The age-adjusted incidence (AAI) was calculated using the SEER*Stat Software version 8.3.6. The Kaplan–Meier method was used to draw survival curves and differences among them were compared by the log-rank test. Cox proportional hazards models were used to evaluate factors that had independent predictive effects on the overall survival. Results The AAI of pancreatic ACC was on the rise with the mean age at diagnosis of 63.79±14.79 years. Most patients (15.9%) had poorer differentiated tumors. The patients presented with distant stage were 54.4% compared with 53.1% between 1988 and 2003. The 1-, 2-, and 5-years survival rates for pancreatic ACC patients were 53.5%, 34.6%,17.5%, respectively (compared with 78.5%, 67.0%, and 42.8%, between 1988 and 2003). The multivariate COX analysis showed that the patient's age, surgery, chemotherapy, and summary stage, but not marital status were independent prognosis factors for ACC. Conclusions Pancreatic ACC is a highly malignant tumor with an increasing incidence in recent years. The rate of distant metastasis is increasing and the survival rate is worse than in the past, suggesting that it may require more aggressive treatment and follow-up. Surgery, radiotherapy, and chemotherapy are all effective treatments, but prospective studies are still needed to verify them.

345 Predictors of Survival in 865 Patients with Acinar Cell Carcinoma of the Pancreas: Survival After Surgery Compared to Ductal Adenocarcinoma

2008 ◽  
Vol 134 (4) ◽  
pp. A-846-A-847
Author(s):  
C. Max Schmidt ◽  
Jesus M. Matos ◽  
David J. Bentrem ◽  
Mark S. Talamonti ◽  
Keith D. Lillemoe ◽  
...  
Keyword(s):  
Cell Carcinoma ◽  
Acinar Cell ◽  
Acinar Cell Carcinoma ◽  
Ductal Adenocarcinoma ◽  
Carcinoma Of The Pancreas

Pancreatic Acinar Cell Carcinoma with Germline BRCA2 Mutation and Severe Pancreatic Panniculitis: A Case Report

2021 ◽  
pp. 1-4
Author(s):  
Lena Dreikhausen ◽  
Nadine Schulte ◽  
Sebastian Belle ◽  
Philip Weidner ◽  
Johannes Moersdorf ◽  
...  
Keyword(s):  
Cell Carcinoma ◽  
Acinar Cell ◽  
Brca2 Mutation ◽  
Susceptibility Gene ◽  
Acinar Cell Carcinoma ◽  
Pancreatic Acinar Cell ◽  
Ductal Adenocarcinoma ◽  
Breast Cancer Susceptibility Gene ◽  
Pancreatic Acinar Cell Carcinoma ◽  
Pancreatic Panniculitis

Pancreatic acinar cell carcinoma (ACC) is a rare malignant disease that displays distinct differences to pancreatic ductal adenocarcinoma. Here, we report the case of a patient with ACC and underlying breast cancer susceptibility gene 2 (BRCA2) germline mutation that developed severe pancreatic panniculitis (PP) during the course of the disease. The patient received a multimodal therapy including surgery, systemic chemotherapy, and targeted therapy with the PARP inhibitor olaparib, resulting in an overall survival of 47 months. Findings from this case are compared to the current knowledge on management of ACC and paraneoplastic PP.

Acinar Cell Carcinoma of the Pancreas in the United States: Prognostic Factors and Comparison to Ductal Adenocarcinoma

2008 ◽  
Vol 12 (12) ◽  
pp. 2078-2086 ◽  
Author(s):  
C. Max Schmidt ◽  
Jesus Manuel Matos ◽  
David J. Bentrem ◽  
Mark S. Talamonti ◽  
Keith D. Lillemoe ◽  
...  
Keyword(s):  
United States ◽  
Prognostic Factors ◽  
Cell Carcinoma ◽  
Acinar Cell ◽  
Acinar Cell Carcinoma ◽  
The United States ◽  
Ductal Adenocarcinoma ◽  
Carcinoma Of The Pancreas

Acinar cell carcinoma of the pancreas: a rare disease with different diagnostic and therapeutic implications than ductal adenocarcinoma

2016 ◽  
Vol 142 (12) ◽  
pp. 2585-2591 ◽  
Author(s):  
Stephan Kruger ◽  
Michael Haas ◽  
Philipp Johannes Burger ◽  
Steffen Ormanns ◽  
Dominik Paul Modest ◽  
...  
Keyword(s):  
Cell Carcinoma ◽  
Rare Disease ◽  
Acinar Cell ◽  
Acinar Cell Carcinoma ◽  
Ductal Adenocarcinoma ◽  
Carcinoma Of The Pancreas ◽  
Therapeutic Implications

scholarly journals Clinical Characteristics of Resected Acinar Cell Carcinoma of the Pancreas: A Korean Multi-Institutional Study

Cancers ◽  
2021 ◽  
Vol 13 (20) ◽  
pp. 5095
Author(s):  
Sang Hyun Shin ◽  
Ho Kyoung Hwang ◽  
Jin-Young Jang ◽  
Hongbeom Kim ◽  
Sang Jae Park ◽  
...  
Keyword(s):  
Lymph Node ◽  
Cell Carcinoma ◽  
Clinical Outcomes ◽  
Acinar Cell ◽  
Large Scale ◽  
Regional Lymph Node ◽  
Treatment Strategies ◽  
Acinar Cell Carcinoma ◽  
Ductal Adenocarcinoma ◽  
Male Predominance

Given the rare incidence of pancreatic acinar cell carcinoma (PACC), its post-resection clinical outcomes remain unclear. Treatment strategies for PACC have relied on those of pancreatic ductal adenocarcinoma (PDAC). The present study retrospectively investigated clinicopathologic characteristics of resected PACC registered in the Korea Tumor Registry System Biliary Pancreas database. Among 59 patients with a mean age of 59.2 years and a male predominance (83.1%), 43, 5, 7, and 4 had pure PACC, ductal differentiations, mixed neuroendocrine carcinomas, and intraductal and papillary variants, respectively. The mean tumor size was 4.6 cm, consisting of eight at T1, 26 at T2, and 25 at T3 stages. Metastasis to regional lymph node was identified in 15 (25.4%) patients. Thirty-one (52.5%) patients received adjuvant therapy. Five-year survival rate was 57.4%. The median survival was 78.8 months. In survival comparison according to the stage with AJCC system, N stage (lymph node metastasis), but not T stage, showed significant differences (p = 0.027). Resected PACC appeared to have clinical outcomes distinct from those of PDAC in this nationwide study. Therefore, large-scale multinational studies are needed to overcome the rarity of PACC and to establish an appropriate treatment strategies and staging system.

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