% IMPORTANT: The following is UTF-8 encoded. This means that in the presence % of non-ASCII characters, it will not work with BibTeX 0.99 or older. % Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or % “biber”. @ARTICLE{Lange:303976, author = {S. Lange and H. Lisiecki and S. Kreutzfeldt$^*$ and C. Heining$^*$ and L. Weiss and C. B. Westphalen$^*$ and A. Stenzinger and D. Hübschmann$^*$ and M. Jesinghaus and H. Glimm$^*$ and S. Fröhling$^*$ and N. Pfarr and A. M. Schlitter$^*$}, title = {{P}recision oncology for advanced-stage adenocarcinoma of the appendix: comprehensive molecular characterisation identifies actionable lesions and potential predictive biomarkers.}, journal = {BMJ open gastroenterology}, volume = {12}, number = {1}, issn = {2054-4774}, address = {London}, publisher = {BMJ Publishing Group}, reportid = {DKFZ-2025-01745}, pages = {e001671}, year = {2025}, abstract = {Appendiceal adenocarcinoma is a rare cancer with very limited therapeutic options. We aimed to determine whether molecular profiling of advanced appendiceal adenocancer can identify actionable therapeutic alterations.We retrospectively analysed cohorts from two large German precision oncology programmes. Patient records and pathology reports from 19 patients with advanced appendiceal adenocarcinoma who were enrolled between 2015 and 2021 were included in this study. We report the molecular features, the resulting molecular tumour board recommendations and their clinical implementation.In $95\%$ of the tumours, at least one potentially actionable alteration was identified, including mutations in ATM, PIK3CA and AKT1. An elevated tumour mutational burden was identified in $26\%$ of the tumours. A total of $74\%$ of all patients received a molecularly driven treatment recommendation, of which 2 $(11\%)$ received the recommended therapy.Molecular profiling of appendiceal adenocarcinomas revealed potentially actionable alterations in a number of cases.}, keywords = {Humans / Appendiceal Neoplasms: genetics / Appendiceal Neoplasms: pathology / Appendiceal Neoplasms: therapy / Female / Male / Adenocarcinoma: genetics / Adenocarcinoma: pathology / Adenocarcinoma: therapy / Adenocarcinoma: drug therapy / Retrospective Studies / Middle Aged / Precision Medicine: methods / Aged / Biomarkers, Tumor: genetics / Mutation / Class I Phosphatidylinositol 3-Kinases: genetics / Adult / Neoplasm Staging / Aged, 80 and over / Ataxia Telangiectasia Mutated Proteins: genetics / Proto-Oncogene Proteins c-akt: genetics / Germany / CANCER (Other) / COLORECTAL NEOPLASIA (Other) / MOLECULAR PATHOLOGY (Other) / Biomarkers, Tumor (NLM Chemicals) / Class I Phosphatidylinositol 3-Kinases (NLM Chemicals) / PIK3CA protein, human (NLM Chemicals) / Ataxia Telangiectasia Mutated Proteins (NLM Chemicals) / ATM protein, human (NLM Chemicals) / Proto-Oncogene Proteins c-akt (NLM Chemicals)}, cin = {B340 / MU01 / HD01 / W015 / DD01}, ddc = {610}, cid = {I:(DE-He78)B340-20160331 / I:(DE-He78)MU01-20160331 / I:(DE-He78)HD01-20160331 / I:(DE-He78)W015-20160331 / I:(DE-He78)DD01-20160331}, pnm = {312 - Funktionelle und strukturelle Genomforschung (POF4-312)}, pid = {G:(DE-HGF)POF4-312}, typ = {PUB:(DE-HGF)16}, pubmed = {pmid:40840958}, doi = {10.1136/bmjgast-2024-001671}, url = {https://inrepo02.dkfz.de/record/303976}, }