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.

scholarly journals Prolonged Survival in a Patient with a Pancreatic Acinar Cell Carcinoma

2015 ◽  
Vol 8 (3) ◽  
pp. 447-450 ◽  
Author(s):  
Anne Ploquin ◽  
Capucine Baldini ◽  
Perrine Vuagnat ◽  
Samira Makhloufi ◽  
Christophe Desauw ◽  
...  
Keyword(s):  
Cell Carcinoma ◽  
Acinar Cell ◽  
Case Reports ◽  
Brca2 Mutation ◽  
Complete Response ◽  
Acinar Cell Carcinoma ◽  
Pancreatic Acinar Cell ◽  
Rare Entity ◽  
Pancreatic Acinar Cell Carcinoma ◽  
Multiple Metastases

Pancreatic acinar cell carcinoma (ACC) is a rare entity. Herein we present the case of a 50-year-old male patient with an unlimited mass on the pancreatic corpus and tail with peripancreatic effusion and multiple metastases in the liver and spleen. A liver biopsy showed a pancreatic ACC. The patient received 9 cycles of gemcitabine plus oxaliplatin (GEMOX regimen), which had to be stopped because of a persistent grade 2 neuropathy. A CT scan showed complete response after 14 years. At the age of 61 years, a localized prostatic cancer was diagnosed, treated by prostatectomy. The patient carried a BRCA2 mutation. None of the precedent case reports describe a chemosensibility to the GEMOX regimen. In spite of the lack of study in these patients, chemotherapy with oxaliplatin seems to be the most effective. Long survival can be expected.

scholarly journals Targeting the NTRK Fusion Gene in Pancreatic Acinar Cell Carcinoma: A Case Report and Review of the Literature

2021 ◽  
Vol 19 (1) ◽  
pp. 10-15
Author(s):  
Medhavi Gupta ◽  
Christopher Sherrow ◽  
Maghan E. Krone ◽  
Edik M. Blais ◽  
Michael J. Pishvaian ◽  
...  
Keyword(s):  
Case Report ◽  
Cell Carcinoma ◽  
Disease Progression ◽  
Acinar Cell ◽  
Acinar Cell Carcinoma ◽  
Pancreatic Acinar Cell ◽  
Ductal Adenocarcinoma ◽  
Molecular Testing ◽  
Clinical Report ◽  
Pancreatic Acinar Cell Carcinoma

Pancreatic acinar cell carcinoma (PACC) is a rare pancreatic exocrine malignancy. Compared with the more common pancreatic ductal adenocarcinoma (PDAC), PACC is more common in younger White men, has earlier stages and a lower mean age (56 vs 70 years) at the time of presentation, and has a better prognosis. In addition to differences in demographic, histologic, and clinical characteristics, PACC has a genomic profile distinct from PDAC, with only rare mutations in TP53, KRAS, and p16 that are commonly found in PDAC. This case report presents a man aged 81 years who presented with a pancreatic body mass with peripancreatic lymph node enlargement. Biopsy of the mass showed acinar cell carcinoma. The patient underwent upfront surgical resection, followed by one cycle of adjuvant gemcitabine, with stoppage of therapy due to poor tolerance. Lower-dose gemcitabine was reintroduced after disease progression 6 months later. Nab-paclitaxel was added to gemcitabine after 6 cycles because of a continued increase in the size of peripancreatic lymph nodes. Combination chemotherapy was stopped after 4 cycles because of further disease progression with new liver metastasis. Molecular testing showed the presence of an SEL1L-NTRK1 fusion. Targeted therapy was started with the oral neurotrophic tropomyosin receptor kinase (NTRK) inhibitor larotrectinib at a dosage of 100 mg twice daily. At the time of writing, the patient has been on therapy for 13 months with an exceptional radiographic response and has not experienced any grade 3 adverse effects. To our knowledge, this is the first clinical report of an NTRK gene fusion in a patient with PACC. This case study highlights the significance of tumor molecular profiling in patients with pancreatic tumors, especially rare histologies.

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