Pulmonary inflammatory myofibroblastic tumor in children
Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm characterized by mesenchymal spindle cell proliferation with a marked inflammatory infiltrate component. Although the exact etiopathology of pulmonary IMT is still not clear, gene fusions involving anaplastic lymphoma kinase (ALK) or ROS pr...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Termedia Publishing House
2023-12-01
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| Series: | Pediatria Polska |
| Subjects: | |
| Online Access: | https://www.termedia.pl/Pulmonary-inflammatory-myofibroblastic-tumor-in-children,127,51989,1,1.html |
| _version_ | 1827288269904150528 |
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| author | Maria Wawszczak Grażyna Kraj Joanna Lange Renata Langfort |
| author_facet | Maria Wawszczak Grażyna Kraj Joanna Lange Renata Langfort |
| author_sort | Maria Wawszczak |
| collection | DOAJ |
| description | Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm characterized by mesenchymal spindle cell proliferation with a marked inflammatory infiltrate component. Although the exact etiopathology of pulmonary IMT is still not clear, gene fusions involving anaplastic lymphoma kinase (ALK) or ROS proto-oncogene 1, receptor tyrosine kinase (ROS1), neurotrophic tyrosine receptor kinase (NTRK), platelet-derived growth factor receptor (PDGFR) and rearranged during transfection (RET) have been recently detected in immunohistochemical assessment of this lesion. Due to its nonspecific clinical or radiological presentation, the diagnosis of IMT primarily depends on histopathological findings. Surgery remains the mainstay of treatment and provides the best chance to limit recurrence. In unresectable lesions or multifocal/metastatic disease, treatment with chemotherapy or ALK, ROS1, RET and NTRK inhibitors is recommended. The prognosis in children with IMT is generally perceived as favorable, although local invasion and metastasis have been reported. |
| first_indexed | 2024-04-24T11:28:53Z |
| format | Article |
| id | doaj.art-71c09288bd3345ff92bf65d9b06620a4 |
| institution | Directory Open Access Journal |
| issn | 0031-3939 2300-8660 |
| language | English |
| last_indexed | 2024-04-24T11:28:53Z |
| publishDate | 2023-12-01 |
| publisher | Termedia Publishing House |
| record_format | Article |
| series | Pediatria Polska |
| spelling | doaj.art-71c09288bd3345ff92bf65d9b06620a42024-04-10T12:37:53ZengTermedia Publishing HousePediatria Polska0031-39392300-86602023-12-0198432032510.5114/polp.2023.13353551989Pulmonary inflammatory myofibroblastic tumor in childrenMaria WawszczakGrażyna KrajJoanna LangeRenata LangfortInflammatory myofibroblastic tumor (IMT) is a rare neoplasm characterized by mesenchymal spindle cell proliferation with a marked inflammatory infiltrate component. Although the exact etiopathology of pulmonary IMT is still not clear, gene fusions involving anaplastic lymphoma kinase (ALK) or ROS proto-oncogene 1, receptor tyrosine kinase (ROS1), neurotrophic tyrosine receptor kinase (NTRK), platelet-derived growth factor receptor (PDGFR) and rearranged during transfection (RET) have been recently detected in immunohistochemical assessment of this lesion. Due to its nonspecific clinical or radiological presentation, the diagnosis of IMT primarily depends on histopathological findings. Surgery remains the mainstay of treatment and provides the best chance to limit recurrence. In unresectable lesions or multifocal/metastatic disease, treatment with chemotherapy or ALK, ROS1, RET and NTRK inhibitors is recommended. The prognosis in children with IMT is generally perceived as favorable, although local invasion and metastasis have been reported.https://www.termedia.pl/Pulmonary-inflammatory-myofibroblastic-tumor-in-children,127,51989,1,1.htmlinflammatory pseudotumor plasma cell granuloma inflammatory myofibroblastic tumor. |
| spellingShingle | Maria Wawszczak Grażyna Kraj Joanna Lange Renata Langfort Pulmonary inflammatory myofibroblastic tumor in children Pediatria Polska inflammatory pseudotumor plasma cell granuloma inflammatory myofibroblastic tumor. |
| title | Pulmonary inflammatory myofibroblastic tumor in children |
| title_full | Pulmonary inflammatory myofibroblastic tumor in children |
| title_fullStr | Pulmonary inflammatory myofibroblastic tumor in children |
| title_full_unstemmed | Pulmonary inflammatory myofibroblastic tumor in children |
| title_short | Pulmonary inflammatory myofibroblastic tumor in children |
| title_sort | pulmonary inflammatory myofibroblastic tumor in children |
| topic | inflammatory pseudotumor plasma cell granuloma inflammatory myofibroblastic tumor. |
| url | https://www.termedia.pl/Pulmonary-inflammatory-myofibroblastic-tumor-in-children,127,51989,1,1.html |
| work_keys_str_mv | AT mariawawszczak pulmonaryinflammatorymyofibroblastictumorinchildren AT grazynakraj pulmonaryinflammatorymyofibroblastictumorinchildren AT joannalange pulmonaryinflammatorymyofibroblastictumorinchildren AT renatalangfort pulmonaryinflammatorymyofibroblastictumorinchildren |