LaVigne Anna W, Meredith David M, D'Adamo David R, Margalit Danielle N
School of Medicine, Johns Hopkins University, Baltimore, Maryland, USA.
Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
BMJ Case Rep. 2018 Apr 11;2018:bcr-2017-221553. doi: 10.1136/bcr-2017-221553.
We present a challenging case of a previously healthy 23-year-old man who developed an inflammatory myofibroblastic tumour of the hard palate, harbouring a rearrangement of the anaplastic lymphoma kinase (ALK) locus. Despite surgical intervention, radiotherapy and ALK-inhibition therapy, the tumour recurred locally and metastasised to regional lymph nodes, and the patient passed away roughly 9 months after diagnosis from local progression. The rapid progression of this patient's disease and its resistance to treatment demonstrate the potentially aggressive clinical course of inflammatory myofibroblastic tumours. ALK-inhibition therapy was unsuccessful in this ALK-positive tumour, highlighting the need for further investigation of markers predictive of disease progression and treatment response.
我们报告了一例具有挑战性的病例，患者为一名23岁既往健康的男性，其硬腭发生了炎性肌纤维母细胞瘤，伴有间变性淋巴瘤激酶（ALK）基因座重排。尽管进行了手术干预、放射治疗和ALK抑制治疗，但肿瘤仍局部复发并转移至区域淋巴结，患者在诊断后约9个月因局部进展而死亡。该患者疾病的快速进展及其对治疗的抵抗性表明炎性肌纤维母细胞瘤可能具有侵袭性的临床病程。ALK抑制治疗对该ALK阳性肿瘤无效，这凸显了进一步研究预测疾病进展和治疗反应的标志物的必要性。
