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A misleading tumor: A rare form of infantile fibromatosis
Infantile fibromatosis is a rare mesenchymal disorder characterized by the fibrous proliferation of the skin, bone, muscle, and viscera. The clinical features vary from solitary to multicentric forms with similar pathological features. Although the tumor is histologically benign, it is a highly infi...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
SAGE Publications
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9969429/ https://www.ncbi.nlm.nih.gov/pubmed/36860305 http://dx.doi.org/10.1177/2050313X231157485 |
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author | El Ouali, Ibtissam Berrada, Kenza Dokal, Ibrahima Diallo Saouab, Rachida El Fenni, Jamal Salaheddine, Tariq |
author_facet | El Ouali, Ibtissam Berrada, Kenza Dokal, Ibrahima Diallo Saouab, Rachida El Fenni, Jamal Salaheddine, Tariq |
author_sort | El Ouali, Ibtissam |
collection | PubMed |
description | Infantile fibromatosis is a rare mesenchymal disorder characterized by the fibrous proliferation of the skin, bone, muscle, and viscera. The clinical features vary from solitary to multicentric forms with similar pathological features. Although the tumor is histologically benign, it is a highly infiltrating lesion making the prognosis poor for patients with craniofacial involvement affection due to the major risk of nerve vascular and airway compression syndrome. The solitary form of infantile fibromatosis observed in the dermis, subcutis, or fibromatosis tends to occur predominantly in males and typically affects craniofacial deep soft tissues. We present a case of an unusual symptom presentation and a rarely observed location of a solitary fibromatosis form, affecting the muscle of the forearm and infiltrating the bone in a 12-year-old girl. Imaging findings were suggestive of rhabdomyosarcoma, but histopathology set the diagnosis of an infantile fibromatosis. The patient, then, received chemotherapy, and amputation was proposed due to the inextricability of this benign yet aggressive tumor, an option that was refused by her parents. We discuss through this article the clinical, radiological, and pathological features of this benign yet aggressive condition, the potential differential diagnosis, the prognosis, and treatment options substantiated with concrete examples from the literature. |
format | Online Article Text |
id | pubmed-9969429 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | SAGE Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-99694292023-02-28 A misleading tumor: A rare form of infantile fibromatosis El Ouali, Ibtissam Berrada, Kenza Dokal, Ibrahima Diallo Saouab, Rachida El Fenni, Jamal Salaheddine, Tariq SAGE Open Med Case Rep Case Report Infantile fibromatosis is a rare mesenchymal disorder characterized by the fibrous proliferation of the skin, bone, muscle, and viscera. The clinical features vary from solitary to multicentric forms with similar pathological features. Although the tumor is histologically benign, it is a highly infiltrating lesion making the prognosis poor for patients with craniofacial involvement affection due to the major risk of nerve vascular and airway compression syndrome. The solitary form of infantile fibromatosis observed in the dermis, subcutis, or fibromatosis tends to occur predominantly in males and typically affects craniofacial deep soft tissues. We present a case of an unusual symptom presentation and a rarely observed location of a solitary fibromatosis form, affecting the muscle of the forearm and infiltrating the bone in a 12-year-old girl. Imaging findings were suggestive of rhabdomyosarcoma, but histopathology set the diagnosis of an infantile fibromatosis. The patient, then, received chemotherapy, and amputation was proposed due to the inextricability of this benign yet aggressive tumor, an option that was refused by her parents. We discuss through this article the clinical, radiological, and pathological features of this benign yet aggressive condition, the potential differential diagnosis, the prognosis, and treatment options substantiated with concrete examples from the literature. SAGE Publications 2023-02-24 /pmc/articles/PMC9969429/ /pubmed/36860305 http://dx.doi.org/10.1177/2050313X231157485 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page (https://us.sagepub.com/en-us/nam/open-access-at-sage). |
spellingShingle | Case Report El Ouali, Ibtissam Berrada, Kenza Dokal, Ibrahima Diallo Saouab, Rachida El Fenni, Jamal Salaheddine, Tariq A misleading tumor: A rare form of infantile fibromatosis |
title | A misleading tumor: A rare form of infantile
fibromatosis |
title_full | A misleading tumor: A rare form of infantile
fibromatosis |
title_fullStr | A misleading tumor: A rare form of infantile
fibromatosis |
title_full_unstemmed | A misleading tumor: A rare form of infantile
fibromatosis |
title_short | A misleading tumor: A rare form of infantile
fibromatosis |
title_sort | misleading tumor: a rare form of infantile
fibromatosis |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9969429/ https://www.ncbi.nlm.nih.gov/pubmed/36860305 http://dx.doi.org/10.1177/2050313X231157485 |
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