Osteosarcoma of the Pelvis: Clinical Presentation and Overall Survival.

Q2 Medicine Sarcoma Pub Date : 2021-12-06 eCollection Date: 2021-01-01 DOI:10.1155/2021/8027314
Jeffrey Mark Brown, David Matichak, Kyla Rakoczy, John Groundland
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引用次数: 11

Abstract

Introduction: Osteosarcoma is the most common sarcoma of bone. Pelvic osteosarcoma presents a significant therapeutic challenge due to potential late symptom onset, metastatic dissemination at diagnosis, and inherent difficulties of wide surgical resection secondary to the complex and critical anatomy of the pelvis. The rates of survival are well reported for osteosarcoma of the appendicular skeleton, but specific details regarding presentation and survival are less known for osteosarcoma of the pelvis.

Methods: The Surveillance, Epidemiology, and End Results (SEER) program was queried for primary osteosarcoma of the bony pelvis from 2004 to 2015. Cases with Collaborative Staging variables (available after 2004) were analyzed by grade, histologic subtype, surgical intervention, tumor size, tumor extension, and presence of metastasis at diagnosis. The 2-, 5-, and 10-year survival rates were assessed with respect to these variables. The SEER database was then queried for age, tumor size, surgical intervention, metastasis at time of presentation, and survivorship data for patients with primary osteosarcoma of the upper extremity, lower extremity, vertebrae, thorax, and face/skull, and rates for all anatomic locations were then compared to patients with primary pelvic osteosarcoma.

Results: A total of 292 cases of pelvic osteosarcoma were identified from 2004 to 2015 within the database, representing 9.8% of cases among all surveyed primary sites. The most common histologic subtype was osteoblastic osteosarcoma (69.9%), followed by chondroblastic osteosarcoma (22.3%). The majority of cases were high-grade tumors (94.3%), of size >8 cm (72.0%), and with extension beyond the originating bone (74.0%). For the entire pelvic osteosarcoma group, the 2-, 5-, 10-year survival rates were 45.6%, 26.5%, and 21.4%, respectively, which were the poorest among surveyed anatomic sites. The 5-year overall survival was an abysmal 5.3% for patients with metastatic disease at diagnosis, and 37.0% for non-metastatic pelvic osteosarcoma treated with surgery and chemotherapy. When compared to other locations, pelvic osteosarcoma had higher rates of metastatic disease at presentation (33.5%), larger median tumor size (11.0 cm), and older median age at diagnosis (47.5 years). While over 85% of patients with tumors at the extremities received surgery, only 47.4% of pelvic osteosarcomas in this cohort received surgical resection-likely influenced by larger tumor size, sacral involvement, frequency of metastasis, older age, or delayed referral to a sarcoma center.

Conclusion: This study clarifies presenting features and clinical outcomes of pelvic osteosarcomas, which often present with large, high-grade tumors with extracompartmental extension, high likelihood of metastatic disease at diagnosis, and a potential limited ability to be addressed surgically. The survival rates of primary osteosarcoma of the pelvis are poor and are lower than osteosarcomas from other anatomic locations. While acknowledging the influence of metastasis, tumor characteristics, and advanced age on the decision to undergo surgical excision of a pelvic osteosarcoma, the rates of surgical resection are low and highlight the importance of understanding appropriate conditions for oncologic resection of pelvic sarcomas.

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骨盆骨肉瘤:临床表现和总生存率。
骨肉瘤是最常见的骨肉瘤。骨盆骨肉瘤由于其潜在的症状出现较晚,诊断时的转移性传播,以及骨盆复杂而关键的解剖结构导致广泛手术切除的固有困难,给治疗带来了重大挑战。阑尾骨肉瘤的存活率有很好的报道,但骨盆骨肉瘤的具体表现和存活率却鲜为人知。方法:对2004 - 2015年骨盆原发性骨肉瘤的监测、流行病学和最终结果(SEER)项目进行查询。采用协同分期变量(2004年以后可用)的病例根据分级、组织学亚型、手术干预、肿瘤大小、肿瘤扩展和诊断时是否存在转移进行分析。根据这些变量评估2年、5年和10年生存率。然后在SEER数据库中查询上肢、下肢、椎骨、胸部和面部/颅骨原发性骨肉瘤患者的年龄、肿瘤大小、手术干预、出现时的转移和生存数据,并将所有解剖部位的发生率与原发性盆腔骨肉瘤患者进行比较。结果:2004年至2015年,数据库中共发现292例盆腔骨肉瘤,占所有调查原发部位病例的9.8%。最常见的组织学亚型为成骨细胞骨肉瘤(69.9%),其次为成软骨细胞骨肉瘤(22.3%)。大多数病例为高级别肿瘤(94.3%),肿瘤大小> 8cm(72.0%),肿瘤扩展到原骨以外(74.0%)。整个盆腔骨肉瘤组的2年、5年、10年生存率分别为45.6%、26.5%和21.4%,是所调查解剖部位中最低的。诊断时患有转移性疾病的患者的5年总生存率为5.3%,而接受手术和化疗的非转移性骨盆骨肉瘤患者的5年总生存率为37.0%。与其他部位相比,盆腔骨肉瘤在表现时具有更高的转移率(33.5%),肿瘤中位尺寸较大(11.0 cm),诊断时中位年龄较大(47.5岁)。虽然超过85%的四肢肿瘤患者接受了手术,但在该队列中,只有47.4%的盆腔骨肉瘤患者接受了手术切除,这可能受到肿瘤较大、累及骶骨、转移频率、年龄较大或延迟转诊到肉瘤中心的影响。结论:本研究明确了盆腔骨肉瘤的表现特征和临床结果,盆腔骨肉瘤通常表现为大的、高级别的肿瘤并伴有室外延伸,诊断时转移性疾病的可能性很高,并且手术治疗的能力可能有限。骨盆原发性骨肉瘤的存活率较低,低于其他解剖部位的骨肉瘤。虽然认识到转移、肿瘤特征和高龄对盆腔骨肉瘤手术切除的影响,但手术切除的率很低,这突出了了解盆腔骨肉瘤肿瘤切除的适当条件的重要性。
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来源期刊
Sarcoma
Sarcoma Medicine-Radiology, Nuclear Medicine and Imaging
CiteScore
5.00
自引率
0.00%
发文量
15
审稿时长
14 weeks
期刊介绍: Sarcoma is dedicated to publishing papers covering all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology and the clinical sciences of epidemiology, surgery, radiotherapy and chemotherapy. High-quality papers concerning the entire range of bone and soft tissue sarcomas in both adults and children, including Kaposi"s sarcoma, are published as well as preclinical and animal studies. This journal provides a central forum for the description of advances in diagnosis, assessment and treatment of this rarely seen, but often mismanaged, group of patients.
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