Gerressen Marcus, Donner Andreas, Jundt Gernot, Jänicke Siegfried, Smeets Ralf, Riediger Dieter, Ghassemi Alireza
Klinik für Zahn-, Mund-, Kiefer- und Plastische Gesichtschirurgie des Universitätsklinikums der RWTH Aachen, Pauwelsstrasse 30, 52074, Aachen, Germany.
Mund Kiefer Gesichtschir. 2006 Sep;10(5):347-52. doi: 10.1007/s10006-006-0021-9.
Osteosarcoma of the jaw (JOS) constituting 5% to 13% of all osteosarcoma is a locally aggressive malignant mesenchymal tumor with high tendency to local recurrence and the ability to produce tumor osteoid. Compared to osteosarcoma of the remaining skeleton (SOS) JOS metastasizes relatively rarely and relatively late. It differs significantly from SOS in its biological behaviour.
The presented case deals with a 60-year-old male patient suffering from a newly occurred polypous mucosal tumor of the right-sided maxillary alveolar ridge bioptically diagnosed as a granulation tissue polyp first. After transmaxillary resection of the tumor and consultation of a bone tumor reference center the final diagnosis of a high-grade chondroblastic osteosarcoma could be made. The recommended radical resection of the tumor was declined by the patient because of the expected cosmetic consequences. After surgical reduction of the rapidly growing sarcoma chemotherapy according to the COOS/EURO-B.O.S.S. protocol was initiated at a stage when computed tomography showed diffuse metastatic disease to the lungs. Chemotherapy could delay the progress of the disease only temporarily. The patient died from respiratory insufficiency.
In JOS radical surgical resection is the therapy of first choice. For substantiation of the diagnosis and central registration of the cases a bone tumor reference center should be contacted.
颌骨骨肉瘤(JOS)占所有骨肉瘤的5%至13%,是一种具有局部侵袭性的恶性间充质肿瘤,局部复发倾向高,且有产生肿瘤类骨质的能力。与其余骨骼的骨肉瘤(SOS)相比,JOS转移相对较少且较晚。其生物学行为与SOS有显著差异。
本病例为一名60岁男性患者,右侧上颌牙槽嵴出现新的息肉状黏膜肿瘤,活检最初诊断为肉芽组织息肉。在经上颌骨切除肿瘤并咨询骨肿瘤参考中心后,最终诊断为高级别软骨母细胞性骨肉瘤。由于预期的美容后果,患者拒绝了推荐的肿瘤根治性切除术。在对快速生长的肉瘤进行手术减瘤后,当计算机断层扫描显示肺部弥漫性转移疾病时,根据COOS/EURO - B.O.S.S.方案开始化疗。化疗只能暂时延缓疾病进展。患者死于呼吸功能不全。
在JOS中,根治性手术切除是首选治疗方法。为了确诊和病例的集中登记,应联系骨肿瘤参考中心。