Other formats:
BibTeX
LaTeX
RIS
@article{1474924,
author = {Zelinka, Jiří and Blahák, Jiří and Daněk, Zdeněk and Bulik, Oliver},
article_location = {Praha},
article_number = {Suppl. 1},
doi = {http://dx.doi.org/10.14735/amcsnn2018S47},
keywords = {chondroblastic osteosarcoma; Li-Fraumeni syndrome; jaw},
language = {eng},
issn = {1210-7859},
journal = {Česká a slovenská neurologie a neurochirurgie},
title = {Chondroblastic osteosarcoma of maxilla, a patient with Li-Fraumeni syndrome},
url = {http://dx.doi.org/10.14735/amcsnn2018S47},
volume = {81},
year = {2018}
}
TY - JOUR
ID - 1474924
AU - Zelinka, Jiří - Blahák, Jiří - Daněk, Zdeněk - Bulik, Oliver
PY - 2018
TI - Chondroblastic osteosarcoma of maxilla, a patient with Li-Fraumeni syndrome
JF - Česká a slovenská neurologie a neurochirurgie
VL - 81
IS - Suppl. 1
SP - 47-50
EP - 47-50
PB - Česká lékařská společnost J.E. Purkyně
SN - 12107859
KW - chondroblastic osteosarcoma
KW - Li-Fraumeni syndrome
KW - jaw
UR - http://dx.doi.org/10.14735/amcsnn2018S47
N2 - 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.
ER -
ZELINKA, Jiří, Jiří BLAHÁK, Zdeněk DANĚK and Oliver BULIK. Chondroblastic osteosarcoma of maxilla, a patient with Li-Fraumeni syndrome. \textit{Česká a slovenská neurologie a neurochirurgie}. Praha: Česká lékařská společnost J.E. Purkyně, 2018, vol.~81, Suppl. 1, p.~47-50. ISSN~1210-7859. Available from: https://dx.doi.org/10.14735/amcsnn2018S47.
|
