Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment

Inflammatory myofibroblastic tumor(IMT)is a rare neoplasm with intermediate malignancy characterized by a propensity for recurrence but a low metastatic rate.Diagnostic challenges arise from the diverse pathological presentation,variable symptomatology,and lack of different imaging features.However,IMT is identified by the fusion of the anaplastic lymphoma kinase(ALK)gene,which is present in approximately 70%of cases,with various fusion partners,including ran-binding protein 2(RANBP2),which allows confirmation of the diagnosis.While surgery is the preferred approach for localized tumors,the optimal long-term treatment for advanced or metastatic disease is difficult to define.Targeted therapies are crucial for achieving sustained response to treatment within the context of genetic alteration in IMT.Crizotinib,an ALK tyrosine kinase inhibitor(TKI),was officially approved by the US Food and Drug Administration(FDA)in 2020 to treat IMT with ALK rearrangement.However,most patients face resistance and disease progression,requiring consideration of sequential treatments.Combining radiotherapy with targeted therapy appears to be beneficial in this indication.Early promising results have also been achieved with immunotherapy,indicating potential for combined therapy approaches.However,defined recommendations are still lacking.This review analyzes the available research on IMT,including genetic disorders and their impact on the course of the disease,data on the latest targeted therapy regimens and the possibility of developing immunotherapy in this indication,as well as summarizing general knowledge about prognostic and predictive factors,also in terms of resistance to systemic therapy.

Small bowel tumors detected and missed during capsule endoscopy: Single center experience

AIM:To characterize small bowel(SB)tumors detected by capsule endoscopy(CE),and identify missed tumors.METHODS:The study included 145 consecutive patients in whom 150 CEs were performed.Following CE,the medical records of the study population were reviewed.Results of double-or single-balloon enteroscopy performed after CE and the results of surgery in all patients operated on were retrieved.The patients were contacted through telephone interviews or postal mail.In addition,the national cancer registry and the polish clinical gastrointestinal stromal tumor(GIST)Registry were searched to identify missed neoplasms.RESULTS:Indications for CE included overt and occult obscure gastrointestinal bleeding(n=81,53.7%),anemia(n=19,12.7%),malabsorption(n=18,12%),abnormal CB follow through(n=9,6%),abdominal pain(n=7,5%),celiac disease(n=5,3%),neuroendocrine tumor(n=3,2%),Crohn’s disease(n=2,<2%),Peutz-Jeghers syndrome(n=2,<2%),other polyposes(n=2,<2%),and diarrhea(n=2,<2%).The capsule reached the colon in 115(76.6%)examinations.In 150 investigations,CE identified 15SB tumors(10%),14 of which were operated on or treated endoscopically.Malignancies included metastatic melanoma(n=1),adenocarcinoma(n=2),and GIST(n=3).Benign neoplasms included dysplastic Peutz-Jeghers polyps(n=4).Non-neoplastic masses included venous malformation(n=1),inflammatory tumors(n=2),and a mass of unknown histology(n=1).During the follow-up period,three additional SB tumors were found(2 GISTs and one mesenteric tumor of undefined nature).The National Cancer Registry and Polish Clinical GIST Registry revealed no additional SB neoplasms in the post-examination period(follow-up:range 4.2-102.5 mo,median 39 mo).The sensitivity of CE for tumor detection was 83.3%,and the negative predictive value was 97.6%.The specificity and positive predictive value were both 100%.CONCLUSION:Neoplasms may be missed by CE,especially in the proximal SB.In overt obscure gastrointestinal bleeding,complementary endoscopic and/or radiologic diagnostic tests are indicated.