Pawar R V, Hagiwara M, Milla S, Wisoff J, George A E
Department of Radiology, New York University Langone Medical Center, New York University School of Medicine; New York, USA -
Neuroradiol J. 2011 Oct 31;24(5):767-71. doi: 10.1177/197140091102400516. Epub 2011 Oct 24.
Langerhans cell histiocytosis (LCH) encompasses a range of clinical presentations. Pure osseous involvement is referred to as eosinophilic granuloma (EG), whereas systemic involvement can either be classified as Hand-Schuller-Christian disease or Letterer-Siwe syndrome. It is estimated that of the total incidence of LCH (0.5 per 100,000 children per year in the United States), nearly 70% are categorized as EG (1). We describe a case of clinically occult calvarial eosinophilic granuloma brought to medical attention only after a traumatic event led to scalp and epidural hemorrhage at the site of the lesion. Osseous EG initially presents as a painful or tender mass, at times even mimicking osteomyelitis. Computed tomography (CT) reveals a lytic lesion with beveled edges. Magnetic resonance (MR) imaging is often non-specific, but usually demonstrates a marrow-replacing process that is T1 hypointense, T2 hyperintense, with homogenous enhancement. The complex MR imaging findings in our case provided a unique perspective regarding the presentation of EG. Once pathology established eosinophilic granuloma, skeletal survey confirmed the lesion was solitary, despite a compelling history of present illness.
朗格汉斯细胞组织细胞增多症(LCH)涵盖一系列临床表现。单纯骨受累称为嗜酸性肉芽肿(EG),而全身受累可分为汉-许-克病或勒-雪综合征。据估计,在美国,LCH的总发病率为每年每10万名儿童中有0.5例,其中近70%被归类为EG(1)。我们描述了一例临床上隐匿的颅骨嗜酸性肉芽肿病例,该病例仅在一次创伤事件导致病变部位头皮和硬膜外出血后才引起医疗关注。骨嗜酸性肉芽肿最初表现为疼痛或压痛性肿块,有时甚至酷似骨髓炎。计算机断层扫描(CT)显示有斜边的溶骨性病变。磁共振(MR)成像通常无特异性,但通常显示骨髓替代过程,T1加权像呈低信号,T2加权像呈高信号,且均匀强化。我们病例中复杂的MR成像表现为嗜酸性肉芽肿的呈现提供了独特视角。一旦病理确诊为嗜酸性肉芽肿,尽管现病史令人信服,但骨骼检查证实该病变为单发。