朗格汉斯细胞组织细胞增多症伴侵犯脑实质:罕见的表现。

Claudio Sebastián Iglesias Vargas, Tomas Quinzacara Aravena, Ingrid Trujillo Ramos, Sebastián Vigueras Alvares
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引用次数: 0

摘要

本病例是一例罕见的朗格汉斯细胞组织细胞增多症,侵犯脑实质,这是一种罕见的现象,在世界范围内很少有病例记录。本病例的相关性在于对小儿神经肿瘤学病理知识的贡献。一名11岁男孩表现为左额叶体积增大,无神经功能缺损或全身症状。CT和MRI显示侵袭性溶骨病变伴病灶周围水肿和颅内扩张。完全切除病变,免疫组织化学证实朗格汉斯细胞组织细胞增生。化疗开始耐受良好,至今无并发症。考虑到朗格汉斯细胞组织细胞增多症对预后的潜在影响以及缺乏针对这类表现的具体指南,本病例强调了及时诊断和治疗朗格汉斯细胞组织细胞增多症累及大脑的必要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Langerhans cells histiocytosis with invasion into the brain parenchyma: A rare presentation.

This case presents an unusual case of Langerhans cell histiocytosis with invasion into the brain parenchyma, a rare phenomenon documented in few cases worldwide. The relevance of this case lies in the contribution to the knowledge of this pathology in the paediatric neuro-oncologic context. An 11-year-old boy presented with increased volume in the left frontal region, without neurological deficit or systemic symptoms. CT and MRI revealed an aggressive osteolytic lesion with perilesional oedema and intracranial extension. Complete excision of the lesion was performed, confirming Langerhans cell histiocytosis by immunohistochemistry. Chemotherapy was started with good tolerance and no complications so far. This case highlights the need for timely diagnosis and treatment in cases of Langerhans cell histiocytosis with brain involvement, given its potential impact on prognosis and the scarcity of specific guidelines for this type of manifestations.

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