关键词: Anaplastic Lymphoma Kinase (ALK) expression Craniofacial Inflammatory Myofibroblastic Tumors (IMT) Pediatric skull lesion

来  源:   DOI:10.1007/s00381-024-06512-7

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(IMT)代表罕见肿瘤,特别是在小儿头骨中很少见。我们介绍了一个新的病例,一个新生男性,右颞部肿块5厘米,并讨论了IMT的当前诊断和治疗方案。手术切除病变的多学科努力取得了成功,病人的颅骨缺损愈合,没有神经缺陷。IMT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IMT的主要治疗方法。有希望的药物治疗,比如克唑替尼,我们需要进一步研究,以了解IMT管理中的潜在替代方案。
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