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Inflammatory Myofibroblastic Tumors in the Uterus: Childhood-Case Report and Review of the Literature
Inflammatory myofibroblastic tumor (IMT) is a spindle cell neoplasm with low malignant potential, which may appear in different parts of the body. Uterine localization is rare, especially among children. Etiology is unclear, although some authors suggest underlying trauma or distress. A 3.5-year-old...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2020
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7033747/ https://www.ncbi.nlm.nih.gov/pubmed/32117841 http://dx.doi.org/10.3389/fped.2020.00036 |
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author | Etlinger, Péter Kuthi, Levente Kovács, Tamás |
author_facet | Etlinger, Péter Kuthi, Levente Kovács, Tamás |
author_sort | Etlinger, Péter |
collection | PubMed |
description | Inflammatory myofibroblastic tumor (IMT) is a spindle cell neoplasm with low malignant potential, which may appear in different parts of the body. Uterine localization is rare, especially among children. Etiology is unclear, although some authors suggest underlying trauma or distress. A 3.5-year-old girl was treated at our institute for recurring vaginal bleeding without injury or known pathology. Physical examination and laboratory analysis revealed no specific findings, contrast-enhanced MRI found a 25 × 28 × 30 mm-sized inhomogeneous soft tissue mass in the uterus wall, which was excised in toto. Histological examination identified a spindle cell pattern, and the FISH test revealed ALK gene rearrangement, the lesion was defined as an IMT. Six cases were published to date, and their diagnostic methods are not equivocal, CT, and PET CT were preferred instead of MRI. Aggressive therapy seems to be exaggerated according to low recurrence and metastasis occurrence, and crizotinib is proved as good therapeutic agent in those cases. Biopsy and histology has important role in order to distinguish IMT from malignancies completed with FISH examination because ALK positivity strengthens the diagnosis. No lethal outcome was published among children, as our patient is also symptom-free after 3 years. |
format | Online Article Text |
id | pubmed-7033747 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-70337472020-02-28 Inflammatory Myofibroblastic Tumors in the Uterus: Childhood-Case Report and Review of the Literature Etlinger, Péter Kuthi, Levente Kovács, Tamás Front Pediatr Pediatrics Inflammatory myofibroblastic tumor (IMT) is a spindle cell neoplasm with low malignant potential, which may appear in different parts of the body. Uterine localization is rare, especially among children. Etiology is unclear, although some authors suggest underlying trauma or distress. A 3.5-year-old girl was treated at our institute for recurring vaginal bleeding without injury or known pathology. Physical examination and laboratory analysis revealed no specific findings, contrast-enhanced MRI found a 25 × 28 × 30 mm-sized inhomogeneous soft tissue mass in the uterus wall, which was excised in toto. Histological examination identified a spindle cell pattern, and the FISH test revealed ALK gene rearrangement, the lesion was defined as an IMT. Six cases were published to date, and their diagnostic methods are not equivocal, CT, and PET CT were preferred instead of MRI. Aggressive therapy seems to be exaggerated according to low recurrence and metastasis occurrence, and crizotinib is proved as good therapeutic agent in those cases. Biopsy and histology has important role in order to distinguish IMT from malignancies completed with FISH examination because ALK positivity strengthens the diagnosis. No lethal outcome was published among children, as our patient is also symptom-free after 3 years. Frontiers Media S.A. 2020-02-14 /pmc/articles/PMC7033747/ /pubmed/32117841 http://dx.doi.org/10.3389/fped.2020.00036 Text en Copyright © 2020 Etlinger, Kuthi and Kovács. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Pediatrics Etlinger, Péter Kuthi, Levente Kovács, Tamás Inflammatory Myofibroblastic Tumors in the Uterus: Childhood-Case Report and Review of the Literature |
title | Inflammatory Myofibroblastic Tumors in the Uterus: Childhood-Case Report and Review of the Literature |
title_full | Inflammatory Myofibroblastic Tumors in the Uterus: Childhood-Case Report and Review of the Literature |
title_fullStr | Inflammatory Myofibroblastic Tumors in the Uterus: Childhood-Case Report and Review of the Literature |
title_full_unstemmed | Inflammatory Myofibroblastic Tumors in the Uterus: Childhood-Case Report and Review of the Literature |
title_short | Inflammatory Myofibroblastic Tumors in the Uterus: Childhood-Case Report and Review of the Literature |
title_sort | inflammatory myofibroblastic tumors in the uterus: childhood-case report and review of the literature |
topic | Pediatrics |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7033747/ https://www.ncbi.nlm.nih.gov/pubmed/32117841 http://dx.doi.org/10.3389/fped.2020.00036 |
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