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@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 a 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, roč.~81, Suppl. 1, s.~47-50. ISSN~1210-7859. Dostupné z: https://dx.doi.org/10.14735/amcsnn2018S47.
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