Samet Jonathan, Weinstein Joanna, Fayad Laura M
Department of Medical Imaging, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, 225 E. Chicago Ave, Chicago, IL, 60611, USA.
Departments of Pediatrics, Division of Hematology/Oncology/Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, 225 E. Chicago Ave, Chicago, IL, 60611, USA.
Skeletal Radiol. 2016 May;45(5):607-13. doi: 10.1007/s00256-016-2330-x. Epub 2016 Jan 22.
To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities.
The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated (location, size, T1/ T2/post-contrast features, perilesional bone and soft tissue signal, endosteal scalloping, periosteal reaction, soft tissue mass, pathologic fracture). Pre-biopsy radiologic diagnoses were collected from the original clinical reports. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), temperature, and white blood cell count (WBC) were collected at the time of diagnosis when available.
The locations of the LCH lesions included five humerus, four femur, five ilium, one tibia, one clavicle, and three scapula. Lesional size ranged from 1.8 to 7.1 cm, with a mean of 3.6 cm. All lesions demonstrated perilesional bone marrow edema, periosteal reaction, endosteal scalloping, and post-contrast enhancement. An associated soft tissue mass was present in 15/21 (71.4 %). Clinically, the WBC, ESR, and CRP were elevated in 2/14 (14 %), 8/12 (67 %), and 4/10 (40 %) of cases, respectively. Fever was documented in 1/15 (7 %) patients and pain was reported in 15/15 (100 %).
The clinical and radiologic features of LCH in the pelvis and extremities overlap with infection and malignancy, but LCH must be considered in the differential diagnosis, as it routinely presents with aggressive MRI features, including endosteal scalloping, periosteal reaction, perilesional edema, and a soft tissue mass. Furthermore, an unknown skeletal lesion at presentation without aggressive MRI features is unlikely to represent LCH.
评估骨盆和四肢朗格汉斯细胞组织细胞增多症的临床及磁共振成像（MRI）特征。
研究21例经病理证实累及骨盆和四肢的朗格汉斯细胞组织细胞增多症（LCH）患者的MRI及临床特征。评估病变的多个特征（位置、大小、T1/T2/增强后特征、病变周围骨及软组织信号、骨内膜扇贝样改变、骨膜反应、软组织肿块、病理性骨折）。从原始临床报告中收集活检前的放射学诊断。如有数据，收集诊断时的红细胞沉降率（ESR）、C反应蛋白（CRP）、体温及白细胞计数（WBC）。
LCH病变的位置包括5例肱骨、4例股骨、5例髂骨、1例胫骨、1例锁骨和3例肩胛骨。病变大小在1.8至7.1厘米之间，平均为3.6厘米。所有病变均表现为病变周围骨髓水肿、骨膜反应、骨内膜扇贝样改变及增强后强化。15/21（71.4%）存在相关软组织肿块。临床上，病例中白细胞、ESR和CRP升高的分别为2/14（14%）、8/12（67%）和4/10（40%）。1/15（7%）患者有发热记录，15/15（100%）患者有疼痛报告。
骨盆和四肢LCH的临床及放射学特征与感染和恶性肿瘤有重叠，但在鉴别诊断中必须考虑LCH，因为其通常表现出侵袭性的MRI特征，包括骨内膜扇贝样改变、骨膜反应、病变周围水肿及软组织肿块。此外，初诊时无侵袭性MRI特征的不明骨骼病变不太可能为LCH。
