关键词: Conservative Eosinophilic granuloma Histiocytosis Observation Review Skull

Mesh : Humans Eosinophilic Granuloma / diagnostic imaging surgery Conservative Treatment Skull / diagnostic imaging surgery pathology Histiocytosis, Langerhans-Cell / surgery Radiography Remission, Spontaneous

来  源:   DOI:10.1007/s00381-023-05926-z   PDF(Pubmed)

Abstract:
Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based on typical clinical and radiological features. While surgical resection has been the standard treatment for EG, growing evidence favors watchful waiting, as unifocal calvarial lesions appear to frequently undergo spontaneous remission. However, histopathological confirmations of this hypothesis are still very limited.
Methods. Here, we report a case of EG with typical clinical and radiological features which, due to intervening circumstances, was resected in a delayed fashion. Moreover, we perform a systematic review of the literature on conservative management of EG.
In our case, histological examination showed ongoing bone regeneration with no traces of the disease. Through our literature review, we found 47 cases of calvarial EG managed with watchful waiting. No active intervention was required in 43 cases (91%). Four patients (9%) received surgery or chemotherapy due to the persistence/progression of symptoms or family request. Three reports other than ours documented spontaneous disease remission in surgically resected EG upon histopathological examination.
Our report provides further evidence that watchful waiting can be a reasonable option in the management of single calvarial EG.
摘要:
背景:嗜酸性肉芽肿(EG)是朗格汉斯细胞组织细胞增生症的最常见形式,表现为颅骨的单个溶骨性病变。其诊断基于典型的临床和放射学特征。虽然手术切除一直是EG的标准治疗方法,越来越多的证据支持警惕等待,因为单局部颅骨病变似乎经常经历自发缓解。然而,对这一假设的组织病理学确认仍然非常有限。
方法:方法。这里,我们报告了一例具有典型临床和放射学特征的EG,由于干预情况,以延迟的方式切除。此外,我们对EG保守管理的文献进行了系统回顾。
结果:在我们的案例中,组织学检查显示正在进行的骨再生,没有疾病的痕迹。通过我们的文献综述,我们发现47例颅骨EG在观察等待下进行了治疗。43例(91%)不需要积极干预。由于症状的持续/进展或家庭要求,四名患者(9%)接受了手术或化疗。除我们以外的其他三份报告均记录了组织病理学检查后手术切除的EG的自发性疾病缓解。
结论:我们的报告提供了进一步的证据,表明观察等待可能是治疗单头盖骨EG的合理选择。
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