Synapse - Survival outcomes and genetic characteristics of resected pancreatic acinar cell carcinoma

Survival outcomes and genetic characteristics of resected pancreatic acinar cell carcinoma Journal Article

Authors:	Blair, A. B.; Radomski, S. N.; Chou, J.; Liu, M.; Howell, T. C.; Park, W.; O'Reilly, E. M.; Zheng, L.; Balachandran, V. P.; Wei, A. C.; Kingham, T. P.; D'Angelica, M. I.; Drebin, J.; Zani, S.; Blazer, D. G. 3rd; Burkhart, R. A.; Burns, W. R. 3rd; Lafaro, K. J.; Allen, P. J.; Jarnagin, W. R.; Lidsky, M. E.; He, J.; Soares, K. C.
Article Title:	Survival outcomes and genetic characteristics of resected pancreatic acinar cell carcinoma
Abstract:	Background: Pancreatic acinar cell carcinoma (pACC) is a rare neoplasm of the exocrine pancreas. There is a dearth of information about tumor characteristics and patient outcomes. This study describes the clinical characteristics, genetic alterations, and survival outcomes of resected pACC. Patients and methods: Consecutive patients undergoing pancreatectomy for pathologically confirmed pACC from 1999 to 2022 across three high-volume pancreas surgery centers were analyzed. Patient demographics, tumor characteristics, treatment data, and genetic sequencing were obtained through retrospective abstraction. Results: A total of 61 patients with resected pACC were identified. Median overall survival (OS) was 73 months and median recurrence free survival was 22 months. Nine patients underwent resection for oligometastatic disease; median OS was not reached after a median follow-up of 31 months from date of metastasectomy. Adjuvant chemotherapy was administered in 67% of patients with FOLFOX/FOLFIRINOX (5-fluorouracil, leucovorin, oxaliplatin, +/- irinotecan) the most common regimen (58%). Sequencing data were obtained in 47 (77%) patients. A mutation in at least one of three core genes associated with the homologous recombination repair (HRR) pathway (BRCA1, BRCA2, or PALB2) occurred in 26% (n = 12) with BRCA2 the most frequently identified. A mutation in any other "non-core" gene associated with DNA damage repair or the HRR pathway was identified in 45% (n = 21) with a high tumor mutational burden of > 10 mutations per megabase in 13%. Conclusions: Resection of pACC is associated with favorable survival outcomes, even in the setting of oligometastatic disease. Mutations in the HRR pathway are common, providing opportunities for potential targeted therapeutic options.
Keywords:	survival; chemotherapy; neoplasms; adenocarcinoma; acinar cell carcinoma; computed-tomography; features; cohort; deficiency; mutational landscape; homologous repair; dna-repair defect; whole exome sequencing; pancreatic exocrine cancer; brca mutant-cells
Journal Title:	Annals of Surgical Oncology
Volume:	32
Issue:	3
ISSN:	1068-9265
Publisher:	Springer
Date Published:	2025-03-01
Start Page:	1869
End Page:	1878
Language:	English
ACCESSION:	WOS:001362289600001
DOI:	10.1245/s10434-024-16331-4
PROVIDER:	wos
PMCID:	PMC11811437
PUBMED:	39576455
Notes:	POF incorrectly assigns affiliations associated with the number 1 as present address as MSK, they worked on study while at MSK -- The MSK Cancer Center Support Grant (P30 CA008748) is acknowledge in the PDF -- Corresponding authors is MSK author: Kevin C. Soares -- Source: Wos