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

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.

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

Journal of Clinical Oncology ◽

10.1200/jco.2002.02.005 ◽

2002 ◽

Vol 20 (24) ◽

pp. 4673-4678 ◽

Cited By ~ 177

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.

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

PLoS ONE ◽

10.1371/journal.pone.0243164 ◽

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.

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Systematic Review and Case Series Report of Acinar Cell Carcinoma of the Pancreas

Cancer Control ◽

10.1177/107327481602300417 ◽

2016 ◽

Vol 23 (4) ◽

pp. 446-454 ◽

Cited By ~ 19

Author(s):

Evan S. Glazer ◽

Kevin G. Neill ◽

Jessica M. Frakes ◽

Domenico Coppola ◽

Pamela J. Hodul ◽

...

Keyword(s):

Systematic Review ◽

Cell Carcinoma ◽

Acinar Cell ◽

Case Series ◽

Acinar Cell Carcinoma ◽

Carcinoma Of The Pancreas ◽

Case Series Report ◽

Series Report

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Acinar cell carcinoma of the pancreas: is resection justified even in limited metastatic disease?

The American Journal of Surgery ◽

10.1016/j.amjsurg.2010.06.004 ◽

2011 ◽

Vol 202 (1) ◽

pp. 23-27 ◽

Cited By ~ 28

Author(s):

Werner Hartwig ◽

Maike Denneberg ◽

Frank Bergmann ◽

Thilo Hackert ◽

Ulf Hinz ◽

...

Keyword(s):

Cell Carcinoma ◽

Acinar Cell ◽

Metastatic Disease ◽

Acinar Cell Carcinoma ◽

Carcinoma Of The Pancreas ◽

Limited Metastatic Disease

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Acinar cell carcinoma in childhood: A case report of a very rare tumor

Forum of Clinical Oncology ◽

10.2478/fco-2019-0017 ◽

2020 ◽

Vol 11 (3) ◽

pp. 3-8

Author(s):

Apostolos Pourtsidis ◽

Smaragda Papachristidou ◽

Orthodoxos Achilleos ◽

Darius Mirza ◽

Marina Servitzoglou ◽

...

Keyword(s):

Cell Carcinoma ◽

Disease Progression ◽

Acinar Cell ◽

Metastatic Disease ◽

Acinar Cell Carcinoma ◽

Diagnosis And Treatment ◽

Pancreatic Tumors ◽

Pathology Report ◽

Rare Type ◽

Carcinoma Of The Pancreas

Abstract Introduction Pancreatic tumors are reported rarely in childhood and represent an extremely rare entity in Pediatric Oncology. One of the least common types of pediatric pancreatic tumor is acinar cell carcinoma (ACC). We aim to present a rare case of ACC and the difficulties we faced during diagnosis and treatment. Patient and Methods An 8-year old girl presented with jaundice. Workup revealed a tumor originating from the head of the pancreas with multiple metastatic lesions in her liver. Evaluation of tumor markers revealed elevated levels of AFP. Pathology report was indicative of acinar cell carcinoma of the pancreas. Results After consulting the EXPeRT group (European Cooperative Study Group for Pediatric Rare Tumors), chemotherapy was initiated. Partial response was observed after the first 4 courses with decrease of AFP levels. While planning her surgery, AFP elevated and a second-line course of chemotherapy was administered. Our patient underwent Whipple’s Duodenopancreatectomy with partial metastasectomy. Although the postoperative period was uneventful, AFP continued to rise even after postoperative chemotherapy was administered. There were signs of metastatic disease progression. Our patient received a third-line regimen with no improvement. She received local radiotherapy and a next-line chemotherapy course. Local relapse and metastatic disease progression placed our patient in palliative care. She passed away nine months after the initial diagnosis. Conclusions Acinar cell carcinoma of the pancreas is a rare type of pediatric cancer with very challenging diagnosis and treatment. Cooperation at the European level and multicenter management of those rare cases is vital for the optimum outcome.

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