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Biller LH , Ukaegbu C , Dhingra TG , Burke CA , Chertock Y , Chittenden A , Church JM , Koeppe ES , Leach BH , Levinson E , Lim RM , Lutz M , Salo-Mullen E , Sheikh R , Idos G , Kastrinos F , Stoffel E , Weiss JM , Hall MJ , Kalady MF , Stadler ZK , Syngal S , Yurgelun MB
A Multi-Institutional Cohort of Therapy-Associated Polyposis in Childhood and Young Adulthood Cancer Survivors
Cancer Prev Res (Phila). 2020 Feb 12;13(3) :291-298
PMID: 32051178 PMCID: PMC7060102 URL: https://www.ncbi.nlm.nih.gov/pubmed/32051178
AbstractPrior small reports have postulated a link between gastrointestinal polyposis and childhood and young adulthood cancer (CYAC) treatment (therapy-associated polyposis; TAP), but this remains a poorly understood phenomenon. The aim of this study was to describe the phenotypic spectrum of TAP in a multi-institutional cohort. TAP cases were identified from eight high-risk cancer centers. Cases were defined as patients with >/=10 gastrointestinal polyps without known causative germline alteration or hereditary colorectal cancer predisposition syndrome who had a history of prior treatment with chemotherapy and/or radiotherapy for CYAC. A total of 34 TAP cases were included (original CYAC: 27 Hodgkin lymphoma, three neuroblastoma, one acute myeloid leukemia, one medulloblastoma, one nephroblastoma, and one non-Hodgkin lymphoma). Gastrointestinal polyposis was first detected at a median of 27 years (interquartile range, 20-33) after CYAC treatment. A total of 12 of 34 (35%) TAP cases had >/=50 colorectal polyps. A total of 32 of 34 (94%) had >1 histologic polyp type. A total of 25 of 34 (74%) had clinical features suggestive of >/=1 colorectal cancer predisposition syndrome [e.g., attenuated familial adenomatous polyposis (FAP), serrated polyposis syndrome, extracolonic manifestations of FAP, mismatch repair-deficient colorectal cancer, or hamartomatous polyposis] including 8 of 34 (24%) with features of multiple such syndromes. TAP is an apparently acquired phenomenon that should be considered in patients who develop significant polyposis without known causative germline alteration but who have had prior treatment for a CYAC. Patients with TAP have features that may mimic various hereditary colorectal cancer syndromes, suggesting multiple concurrent biologic mechanisms, and recognition of this diagnosis may have implications for cancer risk and screening.
Notes1940-6215 Biller, Leah H ORCID: https://orcid.org/0000-0001-7386-7121 Ukaegbu, Chinedu Dhingra, Tara G Burke, Carol A Chertock, Yana Chittenden, Anuradha Church, James M Koeppe, Erika S ORCID: https://orcid.org/0000-0002-9775-6327 Leach, Brandie H Levinson, Elana Lim, Ramona M Lutz, Megan Salo-Mullen, Erin Sheikh, Rania Idos, Gregory Kastrinos, Fay Stoffel, Elena Weiss, Jennifer M ORCID: https://orcid.org/0000-0002-4927-2133 Hall, Michael J Kalady, Matthew F Stadler, Zsofia K Syngal, Sapna ORCID: https://orcid.org/0000-0001-5487-7471 Yurgelun, Matthew B ORCID: https://orcid.org/0000-0002-6184-9273 Journal Article United States Cancer Prev Res (Phila). 2020 Feb 12. pii: 1940-6207.CAPR-19-0416. doi: 10.1158/1940-6207.CAPR-19-0416.