儿童多灶性骨肉瘤。

La Chirurgia degli organi di movimento Pub Date : 2009-05-01 Epub Date: 2009-04-28 DOI:10.1007/s12306-009-0015-5
Beate Kunze, Steffen Bürkle, Torsten Kluba
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引用次数: 10

摘要

骨肉瘤是儿童时期最常见的原发性恶性骨肿瘤之一,主要累及四肢长骨干骺端。在罕见的病例中,患者在诊断时有多发骨病变,有时没有肺转移。这些发病率为0.5-4%的罕见多灶性骨肉瘤的病理模式尚不清楚,尽管在诊断和治疗方面进行了研究,但预后仍然很差。我们报告两例多灶性同步骨肉瘤。两个孩子发现肿瘤时的年龄都是14岁。多发性骨病变的同步或异时发生,最初未见肺转移。在这两个病例中,治疗包括新辅助化疗、肿瘤手术和辅助化疗。肿瘤对化疗的反应在一个病人中很好,在另一个病人中很差。两例患者均行肿瘤初始r0切除。无病时间为肺转移或复发前1年。近年来,通过化疗和积极手术的联合治疗,多灶性骨肉瘤的预后得到了改善。然而,生存时间仍然很短,似乎与肿瘤对化疗的初始组织学反应有关。
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Multifocal osteosarcoma in childhood.

Osteosarcoma is one of the most common primary malignant bone tumours in childhood, mainly affecting the metaphysis of long extremity bones. In rare cases, patients present at time of diagnosis with multiple bone lesions, sometimes in the absence of pulmonary metastases. The pathology pattern of these multifocal osteosarcomas occurring with a rare incidence of 0.5-4% is not yet clear, and in spite of investigations in diagnosis and therapy, the prognosis is still poor. We report two cases of multifocal synchronous osteosarcoma. The age of both children at the time of tumour detection was 14 years. A synchronous or metachronous occurrence of multiple bone lesions, initially in the absence of pulmonary metastases was seen. In both cases, treatment consisted of neoadjuvant chemotherapy, oncologic surgery and adjuvant chemotherapy. Tumour response to chemotherapy was good in one patient, and poor in the other case. In both patients initial R0-resection of the tumours was performed. The disease-free time was 1 year before detection of pulmonary metastases or relapse. By the combination of chemotherapy and aggressive surgery the prognosis in multifocal osteosarcoma has been improved over the last years. Nevertheless, the survival time is still short and seems to be correlated with the initial histological tumour response to chemotherapy.

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