Tsimberidou A-M, Kantarjian H M, Estey E, Cortes J E, Verstovsek S, Faderl S, Thomas D A, Garcia-Manero G, Ferrajoli A, Manning J T, Keating M J, Albitar M, O'Brien S, Giles F J
Department of Leukemia, The University of Texas, MD Anderson Cancer Center, 1400 Holcombe Boulevard, Houston, TX 77030, USA .
Leukemia. 2003 Jun;17(6):1100-3. doi: 10.1038/sj.leu.2402958.
Granulocytic sarcoma (GS) is an extramedullary tumor composed of immature myeloid cells. The objectives of this study were to describe the frequency, presenting characteristics, and survival in patients with nonleukemic GS by conducting a review of all untreated patients presenting to the MD Anderson Cancer Center between January 1990 and June 2002. In all, 21 patients with nonleukemic GS, 1520 patients with acute myeloid leukemia (AML), and 402 patients with high-risk myelodysplastic syndrome (MDS) were identified. GS occurred in 1.4% of patients with AML, and 1.1% of patients with AML or high-risk MDSs. The median patient age was 57 years (range, 7-81). Among 20 patients with available cytogenetics in tissue and/or bone marrow, six had chromosome 8 abnormalities. The median follow-up of surviving patients is 12 months (range, 7-75). In all, 20 patients were treated. Patients were treated with AML-type chemotherapy (n=16), chemotherapy and radiotherapy (n=3), or radiotherapy alone (n=1). A total of 13 patients (65%) achieved complete remission and one patient (5%) achieved partial remission. The median overall survival was 20 months (range, 1-75), median overall failure-free survival was 12 months (range, 1-75). The median survival of patients with chromosome 8 abnormalities was 12 months compared with 40 months of those without (P=0.17). Novel therapies for patients with GS are required.
粒细胞肉瘤(GS)是一种由未成熟髓样细胞组成的髓外肿瘤。本研究的目的是通过回顾1990年1月至2002年6月期间在MD安德森癌症中心就诊的所有未经治疗的患者,描述非白血病性GS患者的发病率、临床表现和生存率。总共确定了21例非白血病性GS患者、1520例急性髓细胞白血病(AML)患者和402例高危骨髓增生异常综合征(MDS)患者。GS在1.4%的AML患者以及1.1%的AML或高危MDS患者中出现。患者的中位年龄为57岁(范围7 - 81岁)。在20例组织和/或骨髓细胞遗传学资料可用的患者中,6例存在8号染色体异常。存活患者的中位随访时间为12个月(范围7 - 75个月)。共有20例患者接受了治疗。患者接受AML型化疗(n = 16)、化疗联合放疗(n = 3)或单纯放疗(n = 1)。总共13例患者(65%)实现完全缓解,1例患者(5%)实现部分缓解。中位总生存期为20个月(范围1 - 75个月),中位无失败总生存期为12个月(范围1 - 75个月)。8号染色体异常患者的中位生存期为12个月,而无异常患者为40个月(P = 0.17)。GS患者需要新的治疗方法。