Department of Head & Neck tumors and Neuroendocrine tumors, Shanghai Medical College, Fudan University, Shanghai, China.
Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China.
Cancer Res Treat. 2023 Jul;55(3):1001-1010. doi: 10.4143/crt.2022.894. Epub 2023 Mar 3.
Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal malignancy that occurs primarily in children and adolescents. The clinical and pathological features of IMT in adult patients are not well understood.
We retrospectively searched for records of adult patients with IMT at Fudan University Shanghai Cancer Center from 2006 to 2021. Clinicopathological data, treatments, and outcomes were collected and analyzed.
Thirty adult patients with IMT, mostly women (60.0%), were included. The median age of the patients was 38 (21-77). The most common primary site was abdominopelvic region (53.3%), followed by lungs (20.0%). Seven patients had an abdominal epithelioid inflammatory myofibroblast sarcoma (EIMS). The positivity rate of anaplastic lymphoma kinase (ALK) was 81.5% (22/27). Sixteen patients with advanced ALK-positive disease received crizotinib, with an objective response rate (ORR) of 81.3% and a disease control rate of 87.5%. The median progression-free survival was 20.8 months. EIMS was associated with more aggressive behavior; however, the prognosis was similar to that of non-EIMS patients after treatment with an ALK inhibitor. At a median follow-up time of 30 months (95% confidence interval [CI], 13.6 to 46.4), the 5-year overall survival was 77% (95% CI, 66 to 88) in all patients.
Adult IMTs appeared more aggressive, with a higher incidence of recurrence and metastases, and patients with EIMS had more aggressive cases. Treatment with ALK inhibitors resulted in a high ORR and a durable response, which suggested that ALK inhibitors could be used as a first-line treatment option in adult patients with ALK-positive advanced IMT.
炎性肌纤维母细胞瘤（IMT）是一种罕见的间叶性恶性肿瘤，主要发生在儿童和青少年。成人患者的 IMT 的临床和病理特征尚未得到很好的理解。
我们回顾性地搜索了 2006 年至 2021 年在复旦大学附属肿瘤医院就诊的成人 IMT 患者的记录。收集并分析了临床病理数据、治疗方法和结果。
30 例成人 IMT 患者，多数为女性（60.0%），中位年龄为 38 岁（21-77 岁）。最常见的原发部位为腹盆腔（53.3%），其次为肺部（20.0%）。7 例患者为腹部上皮样炎性肌纤维母细胞肉瘤（EIMS）。间变性淋巴瘤激酶（ALK）阳性率为 81.5%（22/27）。16 例晚期 ALK 阳性疾病患者接受克唑替尼治疗，客观缓解率（ORR）为 81.3%，疾病控制率为 87.5%。中位无进展生存期为 20.8 个月。EIMS 与更具侵袭性的行为相关；然而，在接受 ALK 抑制剂治疗后，其预后与非-EIMS 患者相似。在中位随访时间为 30 个月（95%置信区间[CI]，13.6 至 46.4）时，所有患者的 5 年总生存率为 77%（95%CI，66%至 88%）。
成人 IMT 表现出更具侵袭性，复发和转移的发生率更高，EIMS 患者的病例更具侵袭性。ALK 抑制剂治疗可获得高客观缓解率和持久缓解，这表明 ALK 抑制剂可作为成人 ALK 阳性晚期 IMT 的一线治疗选择。
