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儿童和青年成人骨盆骨肉瘤:圣裘德儿童研究医院的经验

Osteosarcoma of the pelvis in children and young adults: the St. Jude Children's Research Hospital experience.

作者信息

Saab Raya, Rao Bhaskar N, Rodriguez-Galindo Carlos, Billups Catherine A, Fortenberry Tamra N, Daw Najat C

机构信息

Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105-2794, USA.

出版信息

Cancer. 2005 Apr 1;103(7):1468-74. doi: 10.1002/cncr.20959.

DOI:10.1002/cncr.20959
PMID:15739209
Abstract

BACKGROUND

Pelvic osteosarcomas are difficult to resect. The authors reviewed their institution's experience with patients who had such tumors to characterize the patients' clinical findings and to assess the impact of surgical resection on outcome.

METHODS

A review was conducted of the records from patients with pelvic osteosarcoma who were treated at the authors' institution between January, 1970 and March, 2004.

RESULTS

Among 442 patients with osteosarcoma, 19 patients (4%) had high-grade tumors arising in the pelvic bones, including the ilium in 15 patients, the pubis in 2 patients, and the sacrum in 2 patients. The median patient age at diagnosis was 16.8 years. Four tumors were secondary to radiation therapy. Five patients had metastases in the lung (n = 4 patients) or bone (n = 1 patient) at diagnosis. Ten tumors were chondroblastic. The median greatest tumor dimension for the 13 tumors with known size was 10 cm. Ten patients had unresectable pelvic tumors, and 9 patients underwent hemipelvectomy (2 internal and 7 external); complete resection with negative margins was achieved in 5 patients. Four patients survived, including one patient who survived with disease. Of the three patients who survived disease-free, one patient underwent complete resection, one patient underwent incomplete resection (nonviable tumor at the soft tissue margin) with a good response to chemotherapy, and one patient with a sacral tumor underwent radiotherapy only for local control. Of the 9 patients who underwent resection, 7 experienced disease recurrence (n = 5 patients) or progression (n = 2 patients) at distant sites and died. All patients with metastatic disease at diagnosis died.

CONCLUSIONS

Pelvic osteosarcomas often were large and unresectable. A high propensity for metastasis contributed to the poor outcome of patients with pelvic osteosarcoma. New therapeutic approaches are needed.

摘要

背景

骨盆骨肉瘤难以切除。作者回顾了其所在机构治疗此类肿瘤患者的经验,以描述患者的临床特征,并评估手术切除对预后的影响。

方法

对1970年1月至2004年3月在作者所在机构接受治疗的骨盆骨肉瘤患者的记录进行回顾。

结果

在442例骨肉瘤患者中,19例(4%)发生于骨盆骨的高级别肿瘤,其中15例发生于髂骨,2例发生于耻骨,2例发生于骶骨。诊断时患者的中位年龄为16.8岁。4例肿瘤继发于放射治疗。5例患者在诊断时出现肺转移(4例)或骨转移(1例)。10例肿瘤为软骨母细胞型。已知大小的13例肿瘤的最大径中位数为10 cm。10例患者的骨盆肿瘤无法切除,9例患者接受了半骨盆切除术(2例为内部半骨盆切除术,7例为外部半骨盆切除术);5例患者实现了切缘阴性的完整切除。4例患者存活,其中1例带瘤存活。在3例无病存活的患者中,1例接受了完整切除,1例接受了不完全切除(软组织切缘为无活力肿瘤)且对化疗反应良好,1例骶骨肿瘤患者仅接受了放疗以控制局部病变。在接受切除的9例患者中,7例出现远处疾病复发(5例)或进展(2例)并死亡。所有诊断时伴有转移的患者均死亡。

结论

骨盆骨肉瘤通常体积较大且无法切除。高转移倾向导致骨盆骨肉瘤患者预后不良。需要新的治疗方法。

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Osteosarcoma of the pelvis in children and young adults: the St. Jude Children's Research Hospital experience.儿童和青年成人骨盆骨肉瘤:圣裘德儿童研究医院的经验
Cancer. 2005 Apr 1;103(7):1468-74. doi: 10.1002/cncr.20959.
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