Dedifferentiated chondrosarcoma: The role of chemotherapy with updated outcomes

Dedifferentiated chondrosarcoma: The role of chemotherapy with updated outcomes
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DOI:
10.2106/00004623-200411000-00008
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发表时间:
2004-11-01
影响因子:
5.3
通讯作者:
Scully, SP
Scully, SP
中科院分区:
医学1区
文献类型:
--
作者:
Dickey, ID;Rose, PS;Scully, SP

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背景资料:关于去分化软骨肉瘤患者的生存率,或者更具体地说,关于化疗的疗效和作用,特别是在现代诊断和治疗模式的时代,很少有发表的数据。本研究旨在探讨影像学和化疗的进步对预后的影响,并作为1986年发表的一项研究的延伸。方法:对1986年至2000年间我院收治的42例去分化软骨肉瘤患者进行回顾性病历分析。研究组包括24名男性和18名女性,平均年龄为66岁。去分化软骨肉瘤的诊断经组织学证实,治疗、辅助治疗和生存数据均来自所有患者的病历。所有患者已被随访了至少24 months.Results:肿瘤被分类,根据系统的肌肉骨骼肿瘤协会,为IIA级(5),LIB级(26),和III级(11)。3名患者仅接受活检,18名患者接受了肢体牺牲手术,21名患者接受了肢体保留手术。在接受切除术的患者组中,手术切缘分为病灶内3例,边缘2例,宽19例,根治性15例。27例患者接受新辅助治疗;其中23例仅接受化疗,2例仅接受放疗,2例接受联合治疗。中位生存期为7.5个月,5年无病生存率为7.1%。根据现有数据,使用化疗的无病生存率没有显着差异(p = 0.54),手术切缘的位置(p = 0.14),组织学亚型(p = 0.87),诊断时的肿瘤分期(p = 0.43),肿瘤大小(p = 0.79),或与肢体牺牲手术相对的肢体保留手术的性能结论:尽管在诊断方式和辅助治疗方面取得了进展,去分化软骨肉瘤的预后仍然很差。虽然在大多数病例中实现了局部控制,但远处疾病仍然是最大的临床挑战,在90%的患者中发展。需要努力继续鼓励早期诊断,并开发有效的辅助疗法,以控制远距离疾病。应重新考虑目前辅助化疗的常规使用及其在该人群中的固有风险。
Background: There are very few published data on the survival of patients with dedifferentiated chondrosarcoma, or, more specifically, on the efficacy and role of chemotherapy, especially in the era of modern diagnostic and treatment modalities. The current study examines the influence of advancements in imaging and chemotherapy on outcome and serves as an extension to a previous study published in 1986.Methods: Forty-two patients with dedifferentiated chondrosarcoma who had presented to our institution between 1986 and 2000 were identified, and a retrospective chart review was performed. The study group included twenty-four men and eighteen women with an average age of sixty-six years. The diagnosis of dedifferentiated chondrosarcoma was verified histologically, and data on treatment, adjuvant therapy, and survival were obtained from the medical records of all patients. All patients had been followed for a minimum of twenty-four months.Results: The tumors were classified, according to the system of the Musculoskeletal Tumor Society, as grade IIA (five), grade LIB (twenty-six), and grade III (eleven). Three patients underwent biopsy only, eighteen had a limb-sacrificing procedure, and twenty-one had a limb-sparing procedure. In the group of patients who underwent resection, the surgical margins were classified as intralesional in three, marginal in two, wide in nineteen, and radical in fifteen. Twenty-seven patients received neoadjuvant therapy; of these, twenty-three received chemotherapy only, two received radiotherapy only, and two received combined therapy. The median survival time was 7.5 months, and the five-year rate of disease-free survival was 7.1%. With the numbers available, there was no significant difference in the rate of disease-free survival with respect to the use of chemotherapy (p = 0.54), the location of surgical margins (p = 0.14), the histological subtype (p = 0.87), the tumor stage at the time of diagnosis (p = 0.43), the tumor size (p = 0.79), or the performance of limb-sparing as opposed to limb-sacrificing procedures (p = 0.42).Conclusions: Despite advances in diagnostic modalities and adjuvant therapies, dedifferentiated chondrosarcoma continues to carry a poor prognosis. While local control is achieved in the majority of cases, distant disease remains the greatest clinical challenge, developing in 90% of patients. Efforts are needed to continue to encourage earlier diagnosis and to develop effective adjuvant therapies for the control of distant disease. The routine use of current adjuvant chemotherapy and its inherent risks in this population should be reconsidered.