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Skorupan et al. J Cancer Metastasis Treat 2023;9:5 Journal of Cancer
DOI: 10.20517/2394-4722.2022.106
Metastasis and Treatment

Review Open Access

Two rare cancers of the exocrine pancreas: to treat
or not to treat like ductal adenocarcinoma?

3
5
1,2
1,4
Nebojsa Skorupan , Shadin Ghabra , J. Alberto Maldonado , Yang Zhang , Christine Alewine 1
1
Laboratory of Molecular Biology, NCI Center for Cancer Research, Bethesda, MD 20892, USA.
2
Medical Oncology Program, NCI Center for Cancer Research, Bethesda, MD 20892, USA.
3
Surgical Oncology Program, NCI Center for Cancer Research, Bethesda, MD 20892, USA.
4
Medical Research Scholars Program, NCI Center for Cancer Research, Bethesda, MD 20892, USA.
5
Gastrointestinal Pathology, Joint Pathology Center, Silver Spring, MD 20910, USA.
Correspondence to: Dr. Christine Alewine, Laboratory of Molecular Biology, NCI Center for Cancer Research, 37 Convent Drive
37/5116B, Bethesda, MD 20892, USA. E-mail: Christine.Alewine@nih.gov
How to cite this article: Skorupan N, Ghabra S, Maldonado JA, Zhang Y, Alewine C. Two rare cancers of the exocrine pancreas:
to treat or not to treat like ductal adenocarcinoma?. J Cancer Metastasis Treat 2023;9:5. https://dx.doi.org/10.20517/2394-
4722.2022.106
Received: 19 Aug 2022 First Decision: 24 Nov 2022 Revised: 7 Dec 2022 Accepted: 13 Feb 2023 Published: 7 Mar 2023
Academic Editor: Marco Falasca Copy Editor: Fangling Lan Production Editor: Fangling Lan

Abstract
Pancreatic cancer is an aggressive malignancy with increasing incidence. Pancreatic ductal adenocarcinoma
(PDAC) accounts for > 90% of pancreatic cancer diagnoses, while other exocrine tumors are much rarer. In this
review, we have focused on two rare cancers of the exocrine pancreas: adenosquamous carcinoma of the pancreas
(ASCP) and pancreatic acinar cell carcinoma (PACC). The latest findings regarding their cellular and molecular
pathology, clinical characteristics, prognosis, and clinical management are discussed. New genetic and
transcriptomic data suggest that ASCP is related to or overlaps with the basal transcriptomic subtype of PDAC.
These tumors are highly aggressive and driven by activated KRAS and MYC expression. Clinical outcomes remain
poor and effective treatments are limited. PACC has no morphologic or genetic resemblance to PDAC and more
favorable outcomes. Early stage PACC patients have improved survival with surgical resection and patients with
advanced disease benefit most from platinum- or fluoropyrimidine-containing chemotherapy. Frequency of
actionable genetic mutations is high in this disease and case reports suggest good outcomes when matched
therapy is given. Dedicated clinical studies examining ASCP and PACC are limited and difficult to accrue. Further
research is needed to define optimal clinical management for these rare diseases.

© The Author(s) 2023. Open Access This article is licensed under a Creative Commons Attribution 4.0
International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, sharing,
adaptation, distribution and reproduction in any medium or format, for any purpose, even commercially, as
long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and
indicate if changes were made.

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