Cho Hyun Jeong, Park Sung Chan, Lee Jong Min, Jung Na Young, Park Jun Bum
Department of Neurosurgery, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea.
Brain Tumor Res Treat. 2024 Apr;12(2):148-151. doi: 10.14791/btrt.2024.0018.
Eosinophilic granuloma (EG), a subtype of Langerhans cell histiocytosis (LCH), the monostotic form, is a rare condition characterized by a solitary bone lesion. It is even more unusual for this condition to be accompanied by an epidural hematoma (EDH). This case is unique in that it is the first to involve delayed EDH following a seizure. We describe a remarkable example of EG accompanied by an EDH and consider the rarity of this comorbidity. A 32-month-old boy developed a rapidly growing skull mass following a minor head injury. During surgical preparation for a biopsy, the patient experienced a single convulsion. Imaging following the seizure revealed an EDH in the vicinity of the mass. The mass was excised and confirmed to be an EG, but with positive margins. The patient underwent chemotherapy after systemic skeletal evaluation, in accordance with the LCH III protocol established by the Histiocytosis Society. EG is a rare neoplasm that typically presents as a painless growth on the skull that gradually enlarges over time. The correlation between EG and EDH is exceedingly uncommon, with only a few documented cases. This case study underscores the significance of considering EG in the differential diagnosis of an expanding cranium mass, even when associated with EDH. Prompt diagnosis and treatment can prevent serious complications and improve patient outcomes.
嗜酸性肉芽肿(EG)是朗格汉斯细胞组织细胞增多症(LCH)的一种亚型,单骨型较为罕见,其特征为孤立性骨病变。而这种病症伴有硬膜外血肿(EDH)则更为罕见。此病例独特之处在于,它是首例在癫痫发作后出现延迟性硬膜外血肿的病例。我们描述了一例伴有硬膜外血肿的嗜酸性肉芽肿的显著病例,并探讨了这种合并症的罕见性。一名32个月大的男孩在轻微头部受伤后出现了迅速生长的颅骨肿块。在进行活检的手术准备过程中,患者发生了一次惊厥。惊厥后成像显示肿块附近有硬膜外血肿。肿块被切除并确诊为嗜酸性肉芽肿,但切缘阳性。根据组织细胞增多症协会制定的LCH III方案,患者在进行全身骨骼评估后接受了化疗。嗜酸性肉芽肿是一种罕见的肿瘤,通常表现为颅骨上无痛性生长,且随时间逐渐增大。嗜酸性肉芽肿与硬膜外血肿之间的关联极为罕见,仅有少数病例记录。本病例研究强调,即使在伴有硬膜外血肿的情况下,在鉴别诊断颅骨肿块扩大时考虑嗜酸性肉芽肿的重要性。及时诊断和治疗可预防严重并发症并改善患者预后。