TY - JOUR
T1 - Molecular and Clinical Features of Pancreatic Acinar Cell Carcinoma
T2 - A Single-Institution Case Series
AU - Balachandran Pillai, Ashwathy
AU - Yousef, Mahmoud
AU - Yousef, Abdelrahman
AU - Alfaro-Munoz, Kristin D.
AU - Smaglo, Brandon G.
AU - Willis, Jason
AU - Wolff, Robert A.
AU - Pant, Shubham
AU - Hurd, Mark W.
AU - Maitra, Anirban
AU - Wang, Huamin
AU - Katz, Matthew Harold G.
AU - Prakash, Laura R.
AU - Tzeng, Ching Wei D.
AU - Snyder, Rebecca
AU - Castelnovo, Luca F.
AU - Chen, Anthony
AU - Kravets, Andrey
AU - Kudriavtseva, Kseniia
AU - Tarasov, Artem
AU - Kryukov, Kirill
AU - Ying, Haoqiang
AU - Shen, John Paul
AU - Zhao, Dan
N1 - Publisher Copyright:
© 2024 by the authors.
PY - 2024/10
Y1 - 2024/10
N2 - 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.
AB - 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.
KW - KRAS
KW - OS
KW - acinar cell carcinoma
KW - immunohistochemistry
KW - pancreatic
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U2 - 10.3390/cancers16193421
DO - 10.3390/cancers16193421
M3 - Article
C2 - 39410042
AN - SCOPUS:85206581620
SN - 2072-6694
VL - 16
JO - Cancers
JF - Cancers
IS - 19
M1 - 3421
ER -