Molecular and Clinical Features of Pancreatic Acinar Cell Carcinoma: A Single-Institution Case Series
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Ashwathy Balachandran Pillai
1,
Mahmoud Yousef
2,
Abdelrahman Yousef
2,
Kristin D. Alfaro-Munoz
2,
Brandon G. Smaglo
2,
Jason Willis
2,
Robert A. Wolff
2,
Shubham Pant
2,
Mark W. Hurd
2,3,
Anirban Maitra
4,5,
Huamin Wang
4,5,
Matthew Harold G. Katz
6,
Laura R. Prakash
6,
Ching-Wei D. Tzeng
6,
Rebecca Snyder
6,
Luca F. Castelnovo
2,
Anthony Chen
2,
Andrey Kravets
7,
Kseniia Kudriavtseva
7,
Artem Tarasov
7,
Kirill Kryukov
7,
Haoqiang Ying
8
,
John Paul Shen
2 and
Dan Zhao
2,*add
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1
Department of Hospital Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA
2
Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA
3
Ahmed Center for Pancreatic Cancer Research, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA
4
Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA
5
Department of Translational Molecular Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA
6
Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA
7
BostonGene Corporation, 100 Beaver St, Waltham, MA 02453, USA
8
Department of Molecular and Cellular Oncology, Division of Basic Science Research, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA
*
Author to whom correspondence should be addressed.
Cancers 2024, 16(19), 3421; https://doi.org/10.3390/cancers16193421
Submission received: 19 August 2024
/
Revised: 18 September 2024
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Accepted: 24 September 2024
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Published: 9 October 2024
(This article belongs to the Special Issue Proteomic and Genomic Profiling of Pancreatic Cancer)
Simple Summary
We present a case series of 16 patients with pancreatic acinar cell carcinoma treated at our institution for whom available molecular information was evaluated. Most of the patients had metastatic disease, and all patients tested for KRAS mutations were KRAS wild type. Five of 12 patients who underwent DNA damage repair gene testing had germline and/or somatic mutations. One patient was found to have RET fusion and responded favorably to selpercatinib for over 42 months. We also include two additional cases who underwent BostonGene testing, including genomic alterations, RNA expression, and tumor microenvironment (TME) features. One of the two cases was found to have NTRK1 fusion. These findings highlight the need for further investigation of acinar cell carcinoma using larger samples to refine treatment strategies for this rare pancreatic cancer.
Abstract
Objectives: Acinar cell carcinoma (ACC) accounts for about 1% of pancreatic cancers. The molecular and clinical features of ACC are less characterized than those of pancreatic ductal adenocarcinoma. Methods: We retrospectively evaluated the clinical and molecular features of ACC patients who underwent germline and/or somatic molecular testing at The University of Texas MD Anderson Cancer Center from 2008 to 2022 and two cases from 2023–2024 who underwent RNA and TME analysis by Boston Gene. Patient information was extracted from our institutional database with the approval of the Institutional Review Board. Results: We identified 16 patients with available molecular testing results. Fourteen patients had metastatic disease, one had borderline resectable disease, and one had localized resectable disease at diagnosis. Fifteen patients were wild type for KRAS (one patient had unknown KRAS status). Somatic/germline mutations of DNA damage repair genes (BRCA1/2, PALB2, and ATM) were present in 5 of 12 patients tested for these genes. One patient was found to have RET fusion and responded favorably to selpercatinib for over 42 months. The median overall survival (OS) was 24 months for patients with metastatic disease. One of the additional two cases who underwent BostonGene testing was found to have NTRK1 fusion. RNA and TME analysis by Boston Gene of the two cases reported immune desert features and relatively lower RNA levels of CEACAM5, CD47, CD74, and MMP1 and higher RNA levels of CDH6 compared with PDAC.
Keywords:
KRAS; immunohistochemistry; acinar cell carcinoma; pancreatic; OS
