Chondroblastic osteosarcoma of maxilla, a patient with Li-Fraumeni syndrome

Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F65269705%3A_____%2F18%3A00070339" target="_blank" >RIV/65269705:_____/18:00070339 - isvavai.cz</a>
Alternative codes found
RIV/00216224:14110/18:00104777
Result on the web
<a href="http://www.csnn.eu/en/czech-slovak-neurology-article/chondroblastic-osteosarcoma-of-maxil-la-a-patient-with-li-fraumeni-syndrome-64067?confirm_rules=1" target="_blank" >http://www.csnn.eu/en/czech-slovak-neurology-article/chondroblastic-osteosarcoma-of-maxil-la-a-patient-with-li-fraumeni-syndrome-64067?confirm_rules=1</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.14735/amcsnn2018S47" target="_blank" >10.14735/amcsnn2018S47</a>

Result language
angličtina
Original language name
Chondroblastic osteosarcoma of maxilla, a patient with Li-Fraumeni syndrome
Original language description
Osteosarcoma of the jaw is a very rare and highly malignant tumor, which could be a manifestation of Li-Fraumeni syndrome - an inherited cancer syndrome characterized by a high frequency and wide spectr UM of early onset neoplasms. Incidence of jaw osteosarcorna is only about 0.07/100,000 per year. The tumor's stage and possibility of complete surgical removal have the biggest impact on patient prognosis. Because rare, osteosarcomas are often misdiagnosed initially, which delays treatment and worsens the prognosis. We present a 32-year-old female patient with Li-Fraumeni syndrome and initially misdiagnosed chondroblastic osteosarcoma. After incomplete resection and early local recurrence, which completely filled the defect after subtotal maxillectorny, we achieved curative response and more than 7 years disease-free survival with chemo-radiotherapy. Furthermore, we describe symptoms, diagnosis, and treatment of this rare disease, and also focus on Li-Fraumeni syndrome. Outcome of this case report disagrees with the widely held tenet that chemo-radiotherapy of osteosarcoma of the jaw is considered only a palliative treatment, unexpected response of some tumors to treatment, and the role of p53 mutations still are not clear and are the object of extensive investigations.
Czech name
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Czech description
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Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30212 - Surgery

Project
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Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year
2018
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

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