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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. 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. [ABSTRACT FROM AUTHOR]

Objective: To investigate the legitimacy of 90-day mortality as a measure of hepatopancreatobiliary quality., Background: The 90-day mortality rate has been increasingly but not universally reported after hepatopancreatobiliary surgery. The legitimacy of this definition as a measure of surgical quality has not been evaluated., Methods: We retrospectively reviewed the causes of all deaths that occurred within 365 postoperative days in patients undergoing hepatectomy (n = 2811) and/or pancreatectomy (n = 1092) from January 1997 to December 2012. The rates of surgery-related, disease-related, and overall mortality within 30 days, within 30 days or during the index hospitalization, within 90 days, and within 180 days after surgery were calculated., Results: Seventy-nine (3%) surgery-related deaths and 92 (3%) disease-related deaths occurred within 365 days after hepatectomy. Twenty (2%) surgery-related deaths and 112 (10%) disease-related deaths occurred within 365 days after pancreatectomy. The overall mortality rates at 99 and 118 days optimally reflected surgery-related mortality after hepatobiliary and pancreatic operations, respectively. The 90-day overall mortality rate was a less sensitive but equivalently specific measure of surgery-related death., Conclusions and Relevance: The 99- and 118-day definitions of postoperative mortality optimally reflected surgery-related mortality after hepatobiliary and pancreatic operations, respectively. However, among commonly reported metrics, the 90-day overall mortality rate represents a legitimate measure of surgical quality.