Department of Neurosurgery, ULSS8 Berica, Vicenza, Italy.
Department of Pathology, ULSS8 Berica, Vicenza, Italy.
Childs Nerv Syst. 2023 Aug;39(8):2221-2227. doi: 10.1007/s00381-023-05926-z. Epub 2023 Mar 20.
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.
嗜酸性肉芽肿（EG）是朗格汉斯细胞组织细胞增生症最常见的形式，表现为颅骨单一溶骨性病变。其诊断基于典型的临床和影像学特征。虽然手术切除一直是 EG 的标准治疗方法，但越来越多的证据倾向于密切观察等待，因为单侧颅骨病变似乎经常自发缓解。然而，这一假设的组织病理学证实仍然非常有限。
方法。在这里，我们报告了一例具有典型临床和影像学特征的 EG 病例，由于介入情况，该病例被延迟切除。此外，我们对 EG 保守治疗的文献进行了系统回顾。
在我们的病例中，组织学检查显示正在进行骨再生，没有疾病的痕迹。通过我们的文献回顾，我们发现了 47 例颅骨 EG 采用观察等待的方法进行治疗。43 例（91%）无需主动干预。由于症状持续或家族要求，4 例患者（9%）接受了手术或化疗。除我们的报告外，还有 3 份报告记录了手术切除的 EG 在组织病理学检查时自发疾病缓解。
我们的报告进一步证明，在单侧颅骨 EG 的治疗中，密切观察等待是一种合理的选择。
