{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,14]],"date-time":"2026-04-14T23:57:29Z","timestamp":1776211049775,"version":"3.50.1"},"reference-count":51,"publisher":"American Society of Clinical Oncology (ASCO)","issue":"9","content-domain":{"domain":["ascopubs.org"],"crossmark-restriction":true},"short-container-title":["JCO Precis Oncol"],"published-print":{"date-parts":[[2025,6]]},"abstract":"<jats:sec>\n            <jats:title>PURPOSE<\/jats:title>\n            <jats:p>Hematologic malignancies (HMs) account for 4%-10% of cancers in individuals with Li-Fraumeni syndrome (LFS), but their phenotypic spectrum and clinical outcomes remain incompletely characterized.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>METHODS<\/jats:title>\n            <jats:p>We conducted a retrospective cohort study at The University of Utah and University of Wisconsin-Madison. Cancer genetics registries were reviewed to identify all unrelated families with LFS seen between 2010 and the present with at least one individual with a pathologically confirmed HM. A literature review was conducted to identify individuals in the published literature with LFS with an HM. HM molecular characteristics, treatment, and outcomes were recorded.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>RESULTS<\/jats:title>\n            <jats:p>Among 121 total families with LFS at our institutions, 17 patients from 16 (13%) families diagnosed with LFS had an HM. A literature review found an additional 83 patients with detailed descriptions, for a total of 99 patients with LFS and an HM. The spectrum of HMs included 10 subtypes with a propensity for lymphoid over myeloid diagnoses. Most HMs did not occur after cytotoxic therapy and often responded to usual sporadic HM regimens, but in a subset, unusual toxicities were encountered, especially after hematopoietic stem-cell transplantation.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>CONCLUSION<\/jats:title>\n            <jats:p>Our large cohort of patients with HM and LFS suggest a broad spectrum of HMs in LFS with more lymphoid than myeloid, more de novo than postcytotoxic therapy, and more favorable outcomes than previous reports.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1200\/po-24-00860","type":"journal-article","created":{"date-parts":[[2025,6,20]],"date-time":"2025-06-20T19:59:16Z","timestamp":1750449556000},"update-policy":"https:\/\/doi.org\/10.1200\/crossmark","source":"Crossref","is-referenced-by-count":2,"title":["Hematologic Malignancy Frequency, Phenotypes, and Outcomes in Li-Fraumeni 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