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

Kód výsledku v 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>

Nalezeny alternativní kódy

RIV/00216224:14110/18:00104777

Výsledek na webu

<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>

Jazyk výsledku

angličtina

Název v původním jazyce

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

Popis výsledku v původním jazyce

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&apos;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.

Název v anglickém jazyce

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

Popis výsledku anglicky

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&apos;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.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

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OECD FORD obor

30212 - Surgery

Projekt

—

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2018

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

Česká a slovenská neurologie a neurochirurgie

ISSN

1210-7859

e-ISSN

—

Svazek periodika

81

Číslo periodika v rámci svazku

Suppl. 1

Stát vydavatele periodika

CZ - Česká republika

Počet stran výsledku

4

Strana od-do

"S47"-"S50"

Kód UT WoS článku

000452867800009

EID výsledku v databázi Scopus

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