Cancer Res Treat.  2023 Jul;55(3):1001-1010. 10.4143/crt.2022.894.

Clinicopathological Analysis and Treatment of Adult Patients with Inflammatory Myofibroblastic Tumor: A 15-Year Single- Center Study

• Liu X
• Gong C
• Zhang J
• Feng W
• Guo Y
• Sang Y
• Wang C
• Chen Y
• Wang J
• Yu L
• Zhang X
• Luo Z

• KMID: 2544180
• DOI: http://doi.org/10.4143/crt.2022.894

Abstract

Purpose
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.
Materials and Methods
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.
Results
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.
Conclusion
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.

Keyword

Inflammatory myofibroblastic tumor; Adults; Anaplastic lymphoma kinase; Prognosis

Figure

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  Fig. 1 The images of a patient with metastatic uterine inflammatory myofibroblastic tumor harboring IGFBP5-ALK rearrangement who achieved partial response after crizotinib therapy. (A, B) Positron emission tomography/computed tomographic images of a pelvic tumor at baseline. (C, D) Computed tomography images of a pelvic tumor at baseline. (E, F) A pelvic tumor that regressed after 9 months of crizotinib therapy. ALK, anaplastic lymphoma kinase; IGFBP5, insulin like growth factor binding protein 5.

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  Fig. 2 Swimming plot of adult inflammatory myofibroblastic tumor patients. CR, complete response; PD, progression disease; PR, partial response; SD, stable disease.

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  Fig. 3 The overall survival (OS) of the 30 inflammatory myofibroblastic tumor patients. The 1-year, 3-year and 5-year OS rates were 96% (95% confidence interval [CI], 93 to 99), 85% (95% CI, 76 to 93), and 77% (95% CI, 66 to 88), respectively.

Reference

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