Comprehensive review of pancreatic acinar cell carcinoma: epidemiology, diagnosis, molecular features and treatment

Author:

Ikezawa Kenji1ORCID,Urabe Makiko1,Kai Yugo1,Takada Ryoji1ORCID,Akita Hirofumi23,Nagata Shigenori43,Ohkawa Kazuyoshi1

Affiliation:

1. Osaka International Cancer Institute Department of Hepatobiliary and Pancreatic Oncology, , Osaka, Japan

2. Department of Gastroenterological Surgery , , Osaka, Japan

3. Osaka International Cancer Institute , , Osaka, Japan

4. Department of Diagnostic Pathology and Cytology , , Osaka, Japan

Abstract

Abstract Pancreatic acinar cell carcinoma is a rare form (0.2–4.3%) of pancreatic neoplasm with unique clinical and molecular characteristics, which largely differ from pancreatic ductal adenocarcinoma. Pancreatic acinar cell carcinoma occurs more frequently in males and can occur in children. Serum lipase is elevated in 24–58% of patients with pancreatic acinar cell carcinoma. Pancreatic acinar cell carcinomas tend to be large at diagnosis (median tumour size: ~5 cm) and are frequently located in the pancreas head. Radiologically, pancreatic acinar cell carcinoma generally exhibits a solid appearance; however, necrosis, cystic changes and intratumoral haemorrhage can occur in larger lesions. Immunostaining is essential for the definitive diagnosis of pancreatic acinar cell carcinoma. Compared with pancreatic ductal adenocarcinoma, pancreatic acinar cell carcinoma has a more favourable prognosis. Although radical surgery is recommended for patients with pancreatic acinar cell carcinoma who do not have distant metastases, the recurrence rate is high. The effectiveness of adjuvant therapy for pancreatic acinar cell carcinoma is unclear. The response to FOLFIRINOX is generally favourable, and some patients achieve a complete response. Pancreatic acinar cell carcinoma has a different genomic profile compared with pancreatic ductal adenocarcinoma. Although genomic analyses have shown that pancreatic acinar cell carcinoma rarely has KRAS, TP53 and CDKN2A mutations, it has a higher prevalence of homologous recombination-related genes, including BRCA1/2 and ATM, than pancreatic ductal adenocarcinoma, suggesting high sensitivity to platinum-containing regimens and PARP inhibitors. Targeted therapies for genomic alternations are beneficial. Therefore, genetic testing is important for patients with pancreatic acinar cell carcinoma to choose the optimal therapeutic strategy.

Funder

Towa Pharmaceutical and Sumitomo Chemical

ASKA Pharmaceutical

Publisher

Oxford University Press (OUP)

Subject

Cancer Research,Radiology, Nuclear Medicine and imaging,Oncology,General Medicine

Reference155 articles.

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