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小儿颅骨炎症性肌纤维母细胞瘤:1 例罕见病例报告并文献复习。

Pediatric skull inflammatory myofibroblastic tumor: a rare case report and literature review.

机构信息

NJ Craniofacial Center, Morristown, NJ, 07960, USA.

Department of Pathology, Atlantic Health System, Summit, NJ, 07960, USA.

出版信息

Childs Nerv Syst. 2024 Nov;40(11):3829-3835. doi: 10.1007/s00381-024-06512-7. Epub 2024 Jun 25.

Abstract

Inflammatory myofibroblastic tumors (IMTs) represent rare neoplasms, particularly infrequent in the pediatric skull. We present a novel case of a newborn male with a 5 cm right temporal mass and discuss current diagnostic and treatment options for IMTs. A multidisciplinary effort to surgically remove the lesion was successful, and the patient's skull defect healed without neurological deficits. The etiology of IMTs remains elusive, with proposed associations with chromosomal mutations in the anaplastic lymphoma kinase (ALK) gene. Surgical excision remains the primary treatment for IMTs. Promising pharmacological treatments, like Crizotinib, warrant further research into understanding potential alternatives in IMT management.

摘要

炎性肌纤维母细胞瘤(IMTs)是一种罕见的肿瘤,尤其在儿童颅骨中较为少见。我们报告了一例新生儿男性右侧颞部有一个 5 厘米的肿块,并讨论了 IMTs 的当前诊断和治疗选择。多学科手术切除病变的努力取得了成功,患者的颅骨缺损愈合,没有神经功能缺损。IMTs 的病因仍然难以捉摸,与间变性淋巴瘤激酶(ALK)基因的染色体突变有关。手术切除仍然是 IMTs 的主要治疗方法。有前途的药物治疗,如克唑替尼,需要进一步研究,以了解 IMT 治疗的潜在替代方案。

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