Clinical outcomes of patients with resectable pancreatic acinar cell carcinoma

Seo, Seyoung; Yoo, Changhoon; Kim, Kyu-poy; Ryoo, Baek‐Yeol; Chang, Heung-Moon; Hong, Seung‐Mo; Lee, Jae Hoon; Song, Ki Byung; Hwang, Dae Wook; Kim, Ki‐Hun; Hwang, Shin; Kim, Song Cheol

doi:10.1111/1751-2980.12505

Cited by 17 publications

(24 citation statements)

References 28 publications

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“…Interesting, it was shown that patients who were recommended to receive surgery but did not receive operation finally still had favorable prognosis compared with those who had not receive surgical treatment due to the late stages of diseases, showing that apart from surgery, the clinical characteristics themselves had great impact on prognosis of patients with ACC. What is more, similar to other studies 8 , 29 , tumor size was proved not to be an independent prognostic factor for patients with ACC who were more frequently to have large tumors. In addition, there were no significant differences in OS and CSS stratified by tumor size in the present study.…”

Section: Discussionsupporting

confidence: 85%

Nomogram to Predict Cancer-Specific Survival in Patients with Pancreatic Acinar Cell Carcinoma: A Competing Risk Analysis

Cai

Duan

et al. 2018

J. Cancer

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Background: The objective of this study was to evaluate the probability of cancer-specific death of patients with acinar cell carcinoma (ACC) and build nomograms to predict overall survival (OS) and cancer-specific survival (CSS) of these patients.Methods: Data were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. Patients diagnosed with ACC between 2004 and 2014 were retrospectively collected. Cancer-specific mortality and competing risk mortality were evaluated. Nomograms for estimating 1-, 2- and 3-year OS and CSS were established based on Cox regression model and Fine and Grey's model. The precision of the 1-, 2- and 3-year survival of the nomograms was evaluated and compared using the area under receiver operating characteristic (ROC) curve (AUC).Results: The study cohort included 227 patients with ACC. The established nomograms were well calibrated, and had good discriminative ability, with a concordance index (C-index) of 0.742 for OS prediction and 0.766 for CSS prediction. The nomograms displayed better discrimination power than 7th or 8th edition Tumor-Node-Metastasis (TNM) stage systems in training set and validation set for predicting both OS and CSS. The AUC values of the nomogram predicting 1-, 2-, and 3-year OS rates were 0.784, 0.797 and 0.805, respectively, which were higher than those of 7th or 8th edition TNM stage systems. Regard to the prediction of CSS rates, the AUC values of the nomogram were also higher than those of 7th or 8th edition TNM stage systems.Conclusion: We evaluated the 1-, 2- and 3-year OS and CSS in patients with ACC for the first time. Our nomograms showed relatively good performance and could be considered as convenient individualized predictive tools for prognosis.

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Section: Discussionsupporting

confidence: 85%

Nomogram to Predict Cancer-Specific Survival in Patients with Pancreatic Acinar Cell Carcinoma: A Competing Risk Analysis

Cai

Duan

et al. 2018

J. Cancer

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“…Although tumor grade is considered an important prognostic factor for adenocarcinoma [27], we did not find a significant effect of histological grade on OS in patients with ACC (p>0.05). Similar to other studies [12,13,28], tumor size had no significant effect on OS even though ACC unusually presents with larger tumors.…”

Section: Married Patients Had a Better Prognosissupporting

confidence: 86%

“…Although there was a significant improvement in OS in patients undergoing surgery at any stage, the postoperative recurrence rate is as high as 42-72% [8]. Furthermore, the most common pattern of recurrence in ACC is distant metastasis [28], hence the necessity to adopt more active adjuvant therapy strategies after the operation.…”

Section: More Radical Management Methods May Achieve Longer Survivalmentioning

confidence: 99%

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

et al. 2020

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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.

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“…Due to its rarity, there is no standard treatment for PACC. Complete resection is always the preferred option ( 11 ), and chemotherapy often refers to the regimens of colorectal cancer or ductal adenocarcinoma based on their similar genetic changes for those who cannot undergo resection ( 12 ). A retrospective study showed that about 50%–60% patients diagnosed with metastatic PACC responded to a possible combination of gemcitabine, docetaxel, and erlotinib ( 13 ).…”

Section: Discussionmentioning

confidence: 99%

Rapid Response to the Combination of Lenvatinib and Sintilimab in a Pancreatic Acinar Cell Carcinoma Patient With Elevated Alpha-Fetoprotein: A Case Report

et al. 2021

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A 48-year old woman was diagnosed with metastatic pancreatic acinar cell carcinoma (PACC) and with a marked elevation in alpha-fetoprotein (AFP), this being a recognized but uncommon feature of PACC. As she refused chemotherapy, the combined therapy of lenvatinib and sintilimab (lenvatinib 8 mg, orally, qd; and sintilimab 100 mg, intravenous glucose tolerance test, q21d) was given, which conferred significant tumor shrinkage and long progression-free survival (>21 months). This study is the first report and description of a PACC demonstrating favorable response to the combination therapy of an antiangiogenic agent and immunotherapy.

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Clinical outcomes of patients with resectable pancreatic acinar cell carcinoma

Abstract: Compared with ductal adenocarcinoma, resectable pancreatic ACC has a favorable prognosis after surgery. Considering that distant metastasis is the most common pattern of recurrence, further studies are necessary to define the role of adjuvant chemotherapy for improving survival outcomes.

Cited by 17 publications

References 28 publications

Nomogram to Predict Cancer-Specific Survival in Patients with Pancreatic Acinar Cell Carcinoma: A Competing Risk Analysis

Nomogram to Predict Cancer-Specific Survival in Patients with Pancreatic Acinar Cell Carcinoma: A Competing Risk Analysis

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

Rapid Response to the Combination of Lenvatinib and Sintilimab in a Pancreatic Acinar Cell Carcinoma Patient With Elevated Alpha-Fetoprotein: A Case Report

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