Pulmonary Inflammatory Myofibroblastic Tumor


Andre Goy, MD, MS, chief, Lymphoma Division, chairman, John Theurer Cancer Center at Hackensack University Medical Center, discusses a small phase I trial that used crizotinib (Xalkori) to treat ALK-positive pediatric patients with anaplastic large cell lymphoma (ALCL), neuroblastoma, and inflammatory myofibroblastic tumors (IMTs).
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Medicine by Alexandros G. Sfakianakis,Anapafseos 5 Agios Nikolaos 72100 Crete Greece,00302841026182,00306932607174,alsfakia@gmail.com,
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: A Clinical Retrospective Study of 13 Cases
via BioMed Research International
Background. Inflammatory myofibroblastic tumor (IMT), as a mesenchymal tumor, is common in the lung and abdomen but rare in the paranasal sinus and nasopharynx. Objective. This study aimed to summarize the clinical characteristics of IMT in the paranasal sinus and nasopharynx and analyze the relationship between the treatment and the overall survival (OS). Method. The clinical features, treatment, and follow-up data of patients diagnosed with IMT of the paranasal sinus or nasopharynx from 2006 to 2017 were retrospectively analyzed, and the previous literature was reviewed. Results. IMT often presents as an ill-defined soft-tissue mass with bone destruction and invasion of surrounding structures. The treatment methods used in this study were different combinations of surgery, prednisone, radiotherapy, and chemotherapy or observation alone. Three of the 13 patients were lost and the follow-up time of the remaining 10 cases ranged from 2 to 87 months (median, 39 months). Two patients died of the disease; the other eight patients were stable. The 5-year survival rate was 72%. Among the four methods of treatment, only treatment with prednisone was significantly correlated with better OS (P = 0.046). Conclusions. IMT is an intermediate tumor that often mimics malignancy. We are not sure if IMTs in the nasal cavity are more aggressive because of the biology or if the location and local therapy in the head region is more complicated. Radiologic findings help know the extent of the lesion. For unresectable nasal IMT, combined therapy with glucocorticoids, chemotherapy, and radiotherapy is sometimes a better choice. Glucocorticoids are especially recommended as a basic part of the integrated therapy. However, the standard treatment needs further research.
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Video abstract of case report “Refinement of diagnosis and supporting evidence for use of immunotherapy through sequential biopsies in a case of EML4-ALK positive lung cancer” published in the open access journal OncoTargets and Therapy by Song P, Zhang J, Zhang L.
Abstract: In this case report, we describe a tortuous, yet rare, treatment process of the patient. The first biopsy of the patient suggested inflammatory myofibroblastic tumor, ALK (D5F3) positive. Considering the benign progression of the disease, and no indication for surgical resection, oral prednisone was given first. However, the disease rapidly progressed, and a second biopsy revealed a pulmonary sarcomatoid cancer. Since the biopsy was ALK (D5F3) positive, the effect of crizotinib treatment was significant, though crizotinib resistance unfortunately only occurred after 4 months. The third biopsy pathology was performed and confirmed lung adenocarcinoma. After switching to pembrolizumab treatment, the lesions were significantly reduced after four courses of treatment. The current condition of patient persisted in partial response.
Read the full paper here https://www.dovepress.com/refi....nement-of-diagnosis-