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.
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There are lll cases under 40 years old out of 584 stomach carcinoma from Seoul and Pusan Paik hospitals, Korea. It is 19. 0% of the total stomach carcinoma, and male-female ratio is 1 2: l. Under 35 years, it is 10.0%(62 cases), and male-female ratio is 0.9: l. Undifferentiated carcinoma is the predominant type, 76.7%, and differentiated type 23.3%. Proximal parts of stomach (body, fundus, cardia) are affected more, 46.8%, and antrum 38.5%, and body with antrum 14.7%. The present data (10.6%) of the incidence under 35 years old is notably higher than that of Japan (1.7-2.8%) and America (0.49~2.2%). These data of the incidence appear proportionally high with the degree of risk factors of these three countries. There are more undifferentiated or diffusely infiltrating type in the young adults, and the incidence of this type is stable without affected much by the environmental factor. Only differentiated or intestinal type of stomach cancer is variable with the enviromentai risk factor. These facts suggest that enviromental factor is mostly responsible for the development of intestinal type, and hereditary factor for the diffuse type. Based upon these presumptions, it is easily understandable ta find an each different male-female ratio in older and younger age groups (2: 1, 1: 1, respectively). These principal findings can be expected to exist in other human cancers which are developed by known or suspected carcinogenic agents. Leukemia and nasopharyngeal carcinoma are good examples. For, it is speculated that in young age group, hereditary factor is the cause, and in older age group, environmental factor is the cause in the above cancers.