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Xiaolei Hao, Ruie Feng, Yalan Bi, Yuhan Liu, Chunde Li, Tao Lu and Yongji Tian

Erdheim-Chester disease (ECD) is a rare non–Langerhans cell form of histiocytosis that can affect the central nervous system. ECD predominantly affects adults, and only a few pediatric cases have been reported. The co-occurrence of ECD and Langerhans cell histiocytosis (LCH) is exceedingly rare. An 11-year-old boy, who was diagnosed with LCH 7 years previously, presented with multiple giant intracranial lesions. At the time of his initial diagnosis, only one intracranial lesion was observed, and it began to enlarge. Currently, up to 7 intracranial lesions can be observed in this patient. However, the diagnosis of ECD was not confirmed until this most recent open resection. The BRAF V600E mutation was detected in both LCH and ECD lesions. Dabrafenib therapy exhibited dramatic efficacy in this pediatric patient. This case represents the first successful application of dabrafenib in a pediatric patient with intracranial ECD lesions as well as mixed ECD and LCH. In this article, the authors describe the intricate diagnosis and treatment processes in this patient. Recent studies regarding treatment with BRAF inhibitors for neurological involvement in mixed ECD and LCH are also reviewed.

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Xiao-hui Ren, Chun Chu, Chun Zeng, Yong-ji Tian, Zhen-yu Ma, Kai Tang, Lan-bing Yu, Xiang-li Cui, Zhong-cheng Wang and Song Lin

Object

Intracranial epidermoid cysts are rare, potentially curable, benign lesions that are sometimes associated with severe postoperative complications, including hemorrhage. Delayed hemorrhage, defined as one that occurred after an initial unremarkable postoperative CT scan, contributed to most cases of postoperative hemorrhage in patients with epidermoid cyst. In this study, the authors focus on delayed hemorrhage as one of the severe postoperative complications in epidermoid cyst, report its incidence and its clinical features, and analyze related clinical parameters.

Methods

There were 428 cases of intracranial epidermoid cysts that were surgically treated between 2002 and 2008 in Beijing Tiantan Hospital, and these were retrospectively reviewed. Among them, the cases with delayed postoperative hemorrhage were chosen for analysis. Clinical parameters were recorded, including the patient's age and sex, the chief surgeon's experience in neurosurgery, the year in which the operation was performed, tumor size, adhesion to neurovascular structures, and degree of resection. These parameters were compared in patients with and without delayed postoperative hemorrhage to identify risk factors associated with this entity.

Results

The incidences of postoperative hemorrhage and delayed postoperative hemorrhage in patients with epidermoid cyst were 5.61% (24 of 428) and 4.91% (21 of 428), respectively, both of which were significantly higher than that of postoperative hemorrhage in all concurrently treated intracranial tumors, which was 0.91% (122 of 13,479). The onset of delayed postoperative hemorrhage ranged from the 5th to 23rd day after the operation; the median time of onset was the 8th day. The onset manifestation included signs of intracranial hypertension and/or meningeal irritation (71.4%), brain herniation (14.3%), seizures (9.5%), and syncope (4.8%). Neuroimages revealed hematoma in 11 cases and subarachnoid hemorrhage in 10 cases. The rehemorrhage rate was 38.1% (8 of 21). The mortality rate for delayed postoperative hemorrhage was 28.6% (6 of 21). None of the clinical parameters was correlated with delayed postoperative hemorrhage (p > 0.05), despite a relatively lower p value for adhesion to neurovascular structures (p = 0.096).

Conclusions

Delayed postoperative hemorrhage contributed to most of the postoperative hemorrhages in patients with intracranial epidermoid cysts and was a unique postoperative complication with unfavorable outcomes. Adhesion to neurovascular structures was possibly related to delayed postoperative hemorrhage (p = 0.096).

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Xiaolei Hao, Ruie Feng, Yalan Bi, Yuhan Liu, Chunde Li, Tao Lu and Yongji Tian

Erdheim-Chester disease (ECD) is a rare non–Langerhans cell form of histiocytosis that can affect the central nervous system. ECD predominantly affects adults, and only a few pediatric cases have been reported. The co-occurrence of ECD and Langerhans cell histiocytosis (LCH) is exceedingly rare. An 11-year-old boy, who was diagnosed with LCH 7 years previously, presented with multiple giant intracranial lesions. At the time of his initial diagnosis, only one intracranial lesion was observed, and it began to enlarge. Currently, up to 7 intracranial lesions can be observed in this patient. However, the diagnosis of ECD was not confirmed until this most recent open resection. The BRAF V600E mutation was detected in both LCH and ECD lesions. Dabrafenib therapy exhibited dramatic efficacy in this pediatric patient. This case represents the first successful application of dabrafenib in a pediatric patient with intracranial ECD lesions as well as mixed ECD and LCH. In this article, the authors describe the intricate diagnosis and treatment processes in this patient. Recent studies regarding treatment with BRAF inhibitors for neurological involvement in mixed ECD and LCH are also reviewed.