肌肉骨骼髓样肉瘤:41例成人患者的临床、影像学、治疗和预后。

IF 1.3 4区 医学 Q4 RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING
Rajendra Kumar, Sinchun Hwang, Mathew Antony, Raul F P Valenzuela, Meena Kumar
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引用次数: 0

摘要

目的:分析成人白血病患者骨骼肌髓样肉瘤的症状、影像学特征、治疗和预后。材料和方法:回顾性分析41例经活检证实为骨骼肌髓样肉瘤的成人白血病患者的临床症状、影像学特征、治疗和结局。结果:急性粒细胞白血病19例,慢性粒细胞白血病15例。另外5例先前治疗过的白血病患者包括1例慢性髓性白血病,3例骨髓纤维化,1例骨髓增生异常综合征。其余2例为分离性骨髓肉瘤,骨髓正常,无血液学疾病史。仅骨肿瘤29例,仅肌肉肿瘤3例,骨和软组织肿瘤8例,踝关节滑膜内肿瘤1例。71例局灶性骨肿瘤中,溶解性68例,硬化性3例。此外,1例患者出现弥漫性硬化性骨病变,2例患者出现弥漫性溶解/硬化性骨病变。大多数肿瘤无症状,在影像学上偶然发现。局部疼痛、肿块和病理性骨折是最常见的主诉。椎骨和椎旁软组织肿瘤引起神经系统症状。当肌肉肿瘤累及邻近的骨、神经或脊髓时,就会出现症状。13例肌肉肿瘤中仅有3例表现为可触及肿块。这些肌肉骨骼肿瘤的影像学特征是非特异性的。在MRI上,肌肉和溶解性骨的MSs在T1WI上呈低至等强度,在脂肪抑制的T2WI上呈高强度,在对比后脂肪抑制的T1WI上增强。踝关节滑膜髓样肉瘤MRI显示滑膜弥漫性增厚。F-18 FDG PET-CT有助于5例患者的检测、治疗反应监测和治疗后监测。所有患者均接受以阿糖胞苷为基础的全身抗白血病治疗和选择性放疗、手术切除、骨髓移植和/或这些治疗的组合。已知35例死亡患者出现肌肉骨骼MS后的平均生存时间为12.1个月。结论:肌骨骼髓系肉瘤是AML的一种罕见并发症。大多数肿瘤是无症状的。成像,特别是MRI和18-F FDG PET-CT,在检测和监测治疗反应以及治疗后监测中起着至关重要的作用。该病临床预后差,短期生存率低。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Musculoskeletal Myeloid Sarcoma: Clinical, Imaging, Management, and Outcomes in 41 Adult Patients.

Objective: To analyze symptoms, imaging features, management, and outcomes of musculoskeletal myeloid sarcoma in adult leukemic patients.

Materials and methods: This is a retrospective analysis of clinical symptoms, imaging features, management, and outcomes in 41 adult leukemic patients with biopsy-proven myeloid sarcomas of bones and muscles.

Results: Nineteen patients had acute, and 15 had chronic myeloid leukemia. Additional 5 previously treated leukemia patients included 1 with chronic myeloid leukemia, 3 with myelofibrosis, and 1 with myelodysplastic syndrome. The remaining 2 patients had isolated myeloid sarcoma with normal marrow without a history of hematologic disorder. Twenty-nine patients had bone tumors only, 3 muscle tumors only, 8 both bone and soft tissue tumors, and 1 intraarticular synovial tumor of an ankle. Of the 71 focal bone tumors, 68 were lytic and 3 were sclerotic. In addition, diffuse sclerotic bone lesions were present in 1 patient, and diffuse mixed lytic/sclerotic bone lesions in 2 patients. Most tumors were asymptomatic and were discovered incidentally on imaging. Local pain, mass, and pathologic fractures were the most common complaints when present. Vertebral bone and paravertebral soft tissue tumors caused neurological symptoms. Muscle tumors became symptomatic when they involved adjoining bone, nerve, or spinal cord. Only 3 among 13 muscle tumors presented as palpable masses. The imaging features of these musculoskeletal tumors were nonspecific. On MRI, both muscle and lytic bone MSs were hypo-to-iso-intense on T1WI, hyperintense on fat-suppressed T2WI, and enhanced on post-contrast fat-suppressed T1WI. A synovial myeloid sarcoma of the ankle showed diffusely thickened synovium on MRI. F-18 FDG PET-CT was helpful in the detection, monitoring of treatment response, and post-treatment surveillance in 5 patients. All patients were treated with cytarabine-based systemic anti-leukemic treatment and optional radiation, surgical resection, bone marrow transplant, and/or a combination of these. The known mean survival time of 35 dead patients after the appearance of musculoskeletal MS was 12.1 months.

Conclusions: Musculoskeletal myeloid sarcoma, which can occasionally precede it, is a rare complication of AML. Most tumors are asymptomatic. Imaging, particularly MRI and 18-F FDG PET-CT, plays a crucial role in detecting and monitoring treatment response, as well as post-treatment surveillance. The disease has poor clinical outcomes and short-term survival.

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来源期刊
CiteScore
2.50
自引率
0.00%
发文量
230
审稿时长
4-8 weeks
期刊介绍: The mission of Journal of Computer Assisted Tomography is to showcase the latest clinical and research developments in CT, MR, and closely related diagnostic techniques. We encourage submission of both original research and review articles that have immediate or promissory clinical applications. Topics of special interest include: 1) functional MR and CT of the brain and body; 2) advanced/innovative MRI techniques (diffusion, perfusion, rapid scanning); and 3) advanced/innovative CT techniques (perfusion, multi-energy, dose-reduction, and processing).
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