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Modeling Osteosarcoma Using Li-Fraumeni Syndrome Patient-derived Induced Pluripotent Stem Cells

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Li-Fraumeni-associated osteosarcomas: The French experience.

Emilie Saucier¹, Gaëlle Bougeard², Anne Gomez-Mascard³

¹Department of Children and Adolescents Oncology, Gustave Roussy Cancer, Paris-Saclay University, Villejuif, France.

Pediatric Blood & Cancer

|October 10, 2024

Summary

Li-Fraumeni syndrome (LFS)-associated osteosarcomas often occur in young children and affect the axial skeleton and jaw. While standard treatments are effective, a high risk of subsequent cancers significantly impacts patient prognosis.

Keywords:

Li–Fraumeni syndrome TP53 variants chondroblastic osteosarcoma jaw osteosarcoma osteosarcoma osteosarcomatosis periosteal osteosarcoma predisposition

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Area of Science:

Oncology
Genetics
Pediatric Oncology

Background:

Li-Fraumeni syndrome (LFS) is a rare inherited condition predisposing individuals to various cancers.
Osteosarcoma is a common primary bone cancer, particularly in children and young adults.
Germline TP53 mutations are the primary cause of LFS, leading to increased cancer susceptibility.

Purpose of the Study:

To delineate the clinical characteristics and outcomes of osteosarcomas associated with Li-Fraumeni syndrome.
To compare LFS-associated osteosarcomas with unselected osteosarcoma cohorts.

Main Methods:

Retrospective analysis of TP53 germline variant carriers diagnosed with osteosarcoma in France (1980-2019).
Inclusion of 65 osteosarcomas from 52 patients with available clinical and histological data.
Comparison of clinical characteristics with the National Cancer Institute's SEER database.

Main Results:

LFS-associated osteosarcomas occurred more frequently in patients under 10 years old (23% vs. 9%).
Predominant sites included the axial skeleton (16% vs. 10%) and jaw (15% vs. 3%), with a higher prevalence of chondroblastic and periosteal subtypes.
While initial treatment response and progression-free survival were comparable, 5-year event-free survival was lower due to a high incidence of second malignancies (43.4% cumulative risk at 10 years).

Conclusions:

Young age at diagnosis, axial/jaw sites, specific histological subtypes (chondroblastic, periosteal), and multifocal tumors suggest LFS.
Standard osteosarcoma treatments remain effective for the primary tumor.
The high risk of subsequent malignancies in LFS patients necessitates early recognition for tailored management and surveillance.