PDF(Pubmed)
Abstract：
BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor that is prevalent among children and adolescents. Surgery is the most important therapeutic approach for IMT and complete resection is recommended. Although 50% of IMTs show anaplastic lymphoma kinase (ALK) rearrangements, crizotinib has proven an effective therapeutic approach. However, the genetic landscape of this tumor is still not fully understood and treatment options are limited, especially in the majority of ALK-negative tumors.
METHODS: We describe the clinical case of a healthy 18-year-old female in whom a pulmonary nodule was incidentally detected.
METHODS: Following a small increase in the size of the nodule, the patient underwent both 18FDG-PET/CT and 68Ga-PET/CT, resulting in a suspicion of bronchial hamartoma.
METHODS: The patient underwent surgery and a salivary gland-like lung tumor was diagnosed.
RESULTS: After surgery, the patient was referred to our cancer center, where a review of the histology slides gave a final diagnosis of ALK-negative lung IMT. Given the histology, it was decided not to administer adjuvant therapy and the patient was placed in a 3-monthly follow-up program. The patient is still disease-free 2 years post-surgery.
CONCLUSIONS: Although there is no standard of care for the treatment of IMT, identifying genomic alterations could help to redefine the management of patients with negative-ALK disease. Our review of the literature on IMT and other kinase fusions revealed, in addition to ALK rearrangements, the potential association of ROS1, NTRK, RET, or PDGFR beta alterations with the tumor.
METHODS: We describe the clinical case of a healthy 18-year-old female in whom a pulmonary nodule was incidentally detected.
METHODS: Following a small increase in the size of the nodule, the patient underwent both 18FDG-PET/CT and 68Ga-PET/CT, resulting in a suspicion of bronchial hamartoma.
METHODS: The patient underwent surgery and a salivary gland-like lung tumor was diagnosed.
RESULTS: After surgery, the patient was referred to our cancer center, where a review of the histology slides gave a final diagnosis of ALK-negative lung IMT. Given the histology, it was decided not to administer adjuvant therapy and the patient was placed in a 3-monthly follow-up program. The patient is still disease-free 2 years post-surgery.
CONCLUSIONS: Although there is no standard of care for the treatment of IMT, identifying genomic alterations could help to redefine the management of patients with negative-ALK disease. Our review of the literature on IMT and other kinase fusions revealed, in addition to ALK rearrangements, the potential association of ROS1, NTRK, RET, or PDGFR beta alterations with the tumor.
摘要：
背景：炎性肌纤维母细胞瘤（IMT）是一种罕见的间充质肿瘤，在儿童和青少年中普遍存在。手术是IMT最重要的治疗方法，建议完全切除。虽然50％的IMT显示间变性淋巴瘤激酶（ALK）重排，克唑替尼已被证明是一种有效的治疗方法。然而,这种肿瘤的遗传景观仍然没有完全理解，治疗选择有限，尤其是在大多数ALK阴性肿瘤中。
方法：我们描述了一个健康的18岁女性的临床病例，其中偶然发现了一个肺结节。
方法：随着结节大小的小幅增加，患者接受了18FDG-PET/CT和68Ga-PET/CT,导致怀疑支气管错构瘤。
方法：患者接受了手术，诊断为涎腺样肺肿瘤。
结果：手术后，病人被转诊到我们的癌症中心,对组织学切片的回顾给出了ALK阴性肺IMT的最终诊断。鉴于组织学，决定不给予辅助治疗,并对患者进行3个月的随访.患者在手术后2年仍无病。
结论：尽管没有治疗IMT的标准，识别基因组改变有助于重新定义ALK阴性患者的治疗.我们对IMT和其他激酶融合的文献的回顾显示，除了ALK重新安排,ROS1、NTRK、RET,或肿瘤的PDGFRβ改变。
方法：我们描述了一个健康的18岁女性的临床病例，其中偶然发现了一个肺结节。
方法：随着结节大小的小幅增加，患者接受了18FDG-PET/CT和68Ga-PET/CT,导致怀疑支气管错构瘤。
方法：患者接受了手术，诊断为涎腺样肺肿瘤。
结果：手术后，病人被转诊到我们的癌症中心,对组织学切片的回顾给出了ALK阴性肺IMT的最终诊断。鉴于组织学，决定不给予辅助治疗,并对患者进行3个月的随访.患者在手术后2年仍无病。
结论：尽管没有治疗IMT的标准，识别基因组改变有助于重新定义ALK阴性患者的治疗.我们对IMT和其他激酶融合的文献的回顾显示，除了ALK重新安排,ROS1、NTRK、RET,或肿瘤的PDGFRβ改变。
