Definition
Pancreatic acinar cell carcinoma (ACC) is a malignant epithelial neoplasm, which shows acinar cell differentiation by expressing acinar specific products such as trypsin, chymotrypsin, lipase, amylase, and carboxyl ester lipase (CEL).
Clinical Features
Incidence
ACCs are rare neoplasms accounting for about 1–2% of pancreatic neoplasms in adults and about 15% in children. The incidence rate in pediatric population (0–19 years), age adjusted to the 2000 US standard, is 0.003 (La Rosa et al. 2015).
Age
The average age of adult patients is approximately 60 years (range 20–88 years), while in children is 9 years (range 3–16 years).
Sex
Males are more commonly affected, with a male to female ratio of 2.1.
Site
ACCs may arise in any portion of the pancreas, both in adults and children.
Treatment
When possible, the treatment of choice is surgery, followed by adjuvant therapy (Patel et al. 2020). For not resectable ACCs, chemotherapy is the therapeutic standard.
Clinical Symptoms and...
This is a preview of subscription content, log in via an institution to check access.
References and Further Reading
La Rosa, S., Franzi, F., Marchet, S., Finzi, G., Clerici, M., Vigetti, D., Chiaravalli, A. M., Sessa, F., & Capella, C. (2009). The monoclonal anti-BCL10 antibody (clone 331.1) is a sensitive and specific marker of pancreatic acinar cell carcinoma and pancreatic metaplasia. Virchows Archiv, 454, 133–142.
La Rosa, S., Adsay, V., Albarello, L., et al. (2012). Clinicopathologic study of 62 acinar cell carcinomas of the pancreas: Insights into the morphology and immunophenotype and search for prognostic markers. The American Journal of Surgical Pathology, 36, 1782–1795.
La Rosa, S., Sessa, F., & Capella, C. (2015). Acinar cell carcinoma of the pancreas: Overview of clinicopathologic features and insights into the molecular pathology. Frontiers in Medicine, 2, 41.
La Rosa, S., Klimstra, D. S., & Wood, L. D. (2019). Pancreatic acinar cell carcinoma. In WHO Classification of Tumours Editorial Board (Ed.), WHO classification of tumours (Digestive system tumours, 5th ed., pp. 333–336). IARC: Lyon.
Maragliano, R., Vanoli, A., Albarello, L., et al. (2015). ACTH-secreting pancreatic neoplasms associated with Cushing syndrome: Clinicopathologic study of 11 cases and review of the literature. The American Journal of Surgical Pathology, 39, 374–382.
Patel, D. J., Lutfi, W., Sweigert, P., Eguia, E., Abood, G., Knab, L., Kuo, P. C., & Baker, M. S. (2020). Clinically resectable acinar cell carcinoma of the pancreas: Is there a benefit to adjuvant systemic therapy? American Journal of Surgery, 219, 522–526.
Saglietti, C., Schneider, V., Bongiovanni, M., Missiaglia, E., Bisig, B., Dorta, G., Demartines, N., Sempoux, C., & La Rosa, S. (2019). Acinar cell carcinoma of the pancreas with thyroid-like follicular features: First description of a new diagnostic challenging subtype. Virchows Archiv, 475, 789–794.
Seket, B., Saurin, J. C., Scoazec, J. Y., & Partensky, C. (2003). Pancreatic acinar cell carcinoma in a patient with familial adenomatous polyposis. Gastroentérologie Clinique et Biologique, 27, 818–820.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2021 Springer Nature Switzerland AG
About this entry
Cite this entry
La Rosa, S., Sciarra, A. (2021). Acinar Cell Carcinoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5537-1
Download citation
DOI: https://doi.org/10.1007/978-3-319-28845-1_5537-1
Received:
Accepted:
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-28845-1
Online ISBN: 978-3-319-28845-1
eBook Packages: Springer Reference MedicineReference Module Medicine