Acinar cell carcinoma of the pancreas with and without endocrine differentiation

Virlos, Ioannis T.; Papazachariou, I.M.; Wiliamson, R.C.N.

doi:10.1080/136518202760378452

Cited by 16 publications

(11 citation statements)

References 20 publications

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“…Pancreatic carcinomas with acinar differentiation are a very rare pancreatic neoplasm, comprising about 1%-2% of all pancreatic tumors in adults[ 1 - 3 ]. These tumors also occur in children, accounting for 6% of all childhood tumors and more importantly, 15%, of all pediatric pancreatic tumors[ 1 , 2 , 4 - 6 ]. These pancreatic neoplasms include pancreatic acinar cell carcinoma (PACC), pancreatoblastoma, and carcinomas of mixed differentiation.…”

Section: Introductionmentioning

confidence: 99%

Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors

Al‐Hader

Al‐Rohil

Han

et al. 2017

WJG

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Pancreatic carcinomas with acinar differentiation are rare, accounting for 1%-2% of adult pancreatic tumors; they include pancreatic acinar cell carcinoma (PACC), pancreatoblastoma, and carcinomas of mixed differentiation. Patients with PACC have a prognosis better than pancreatic ductal adenocarcinomas but worse than pancreatic neuroendocrine tumors. Reports of overall survival range from 18 to 47 mo. A literature review on PACCs included comprehensive genomic profiling and whole exome sequencing on a series of more than 70 patients as well as other diagnostic studies including immunohistochemistry. Surgical resection of PACC is the preferred treatment for localized and resectable tumors. The efficacy of adjuvant treatment is unclear. Metastatic PACCs are generally not curable and treated with systemic chemotherapy. They are moderately responsive to chemotherapy with different regimens showing various degrees of response in case reports/series. Most of these regimens were developed to treat patients with pancreatic ductal adenocarcinomas or colorectal adenocarcinomas. Review of PACC’s molecular profiling showed a number of gene alterations such as: SMAD4, BRAF, BRCA2, TP53, RB1, MEN1, JAK-1, BRCA-1, BRCA-2, and DNA mismatch repair abnormalities. PACCs had multiple somatic mutations with some targetable with available drugs. Therefore, molecular profiling of PACC should be an option for patients with refractory PACC.

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Section: Introductionmentioning

confidence: 99%

Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors

Al‐Hader

Al‐Rohil

Han

et al. 2017

WJG

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“…Firstly, the SEER database lacks some detailed data such as surgical margin, radiation dose, chemotherapy, and regimen which somehow limited the analysis. Secondly, ACC often incorporates other endocrine components [ 35 ]. Although we excluded patients with acinar cystadenoma, there is no centralized pathologist in the SEER database, hence a possibility of cancer misclassification.…”

Section: Discussionmentioning

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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“… 2 , 4 The Schmid triad of subcutaneous fat necrosis, polyarthralgia, and eosinophilia is only seen in 15% of patients, and is due to lipase hypersecretion by the tumor. 3 , 4 , 6 The classic genetic mutations associated with PDA, for example, the activation of KRAS2 and inactivation of p16/CDKN2A , TP53 , and SMAD4/DPC4 , are very rarely seen. 7 Instead, the mutation profile of acinar cell carcinoma is more comparable to that of pancreatoblastoma, with both tumor types frequently exhibiting allelic loss on chromosome 11p and alterations in the beta-catenin pathway (e.g., APC truncation and beta-catenin activation).…”

Section: Discussionmentioning

confidence: 99%

Neoadjuvant Chemotherapy and Appleby Procedure for Pancreatic Acinar Cell Carcinoma: A Case Report

Jimbo

Batista

Baliff

et al. 2016

Case Reports in Pancreatic Cancer

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Background: Acinar cell carcinoma is a rare form of pancreatic cancer, accounting for 1–2% of all cases of exocrine pancreatic neoplasms in adults. Due to its rarity, no randomized controlled trials have been performed to determine the optimal treatment options. As such, high-quality case reports and case series are needed to help guide clinicians in the management of this deadly disease.Case Presentation: A 56-year-old Caucasian male presenting with abdominal pain and weight loss was diagnosed with stage III acinar cell carcinoma of the pancreatic body with celiac axis involvement. Although initially deemed unresectable, the patient responded favorably to nine cycles of 5-fluorouracil-based neoadjuvant chemotherapy. The tumor was successfully resected through distal pancreatectomy with en bloc splenectomy and en bloc celiac artery resection (Appleby procedure). Final pathology analysis showed negative resection margins and complete chemotherapeutic response within the pancreas, with residual tumor cells detected in only a single peripancreatic lymph node.Conclusion: 5-fluorouracil-based chemotherapy may be a promising option for the neoadjuvant treatment of locally unresectable acinar cell carcinoma. With sufficient expertise, negative surgical resection margins are possible even with vascular involvement. Due to the generally poor prognosis associated with acinar cell carcinoma, such aggressive treatment measures are warranted.

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Acinar cell carcinoma of the pancreas with and without endocrine differentiation

Abstract: Acinar structures are the hallmark of this neoplasm, which carries a better survival rate than ductal cancer. Surgical excision prolongs survival and offers the best chance of cure.

Cited by 16 publications

References 20 publications

Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors

Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors

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

Neoadjuvant Chemotherapy and Appleby Procedure for Pancreatic Acinar Cell Carcinoma: A Case Report

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