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Case report and review of literature: Resection of a huge mediastinal low-grade fibromyxoid sarcoma with neck, axillary, and lung involvement
Low-grade fibromyxoid sarcoma is an extremely rare malignant neoplasm, with an incidence of 0.18 per million, and comprises 0.6% of all soft tissue sarcomas. It has a high recurrence rate and late metastatic spread and is chemotherapy and radiotherapy insensitive. This paper reports a case of an unu...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9632998/ https://www.ncbi.nlm.nih.gov/pubmed/36338657 http://dx.doi.org/10.3389/fsurg.2022.988881 |
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author | Khamashta, Natalie Dalal, Ahmad Alashwas, Mo’men Idkedek, Mayar Abu-Akar, Firas |
author_facet | Khamashta, Natalie Dalal, Ahmad Alashwas, Mo’men Idkedek, Mayar Abu-Akar, Firas |
author_sort | Khamashta, Natalie |
collection | PubMed |
description | Low-grade fibromyxoid sarcoma is an extremely rare malignant neoplasm, with an incidence of 0.18 per million, and comprises 0.6% of all soft tissue sarcomas. It has a high recurrence rate and late metastatic spread and is chemotherapy and radiotherapy insensitive. This paper reports a case of an unusually large mediastinal low-grade fibromyxoid sarcoma in a 55-year-old patient. The tumor was engulfing the main blood vessels of the mediastinum, involving the lung, and extending beyond the chest cavity to involve the cervical and axillary regions. The patient has a 21-year history of frequent surgical resections for lesions that were repeatedly misdiagnosed as neurofibroma. The tumor was successfully resected by a challenging operation that involved mediastinal mass resection, chest wall mass resection, and wedge resection of the left upper lobe of the lung. The deceivingly benign-looking histology of this tumor makes it a commonly misdiagnosed one, requiring careful assessment by pathologists to reach the right diagnosis. Surgical resection with clear margins remains the treatment of choice for these lesions. Due to the behavior of this tumor, once detected and managed, extensive long-term follow-up is always recommended. |
format | Online Article Text |
id | pubmed-9632998 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-96329982022-11-04 Case report and review of literature: Resection of a huge mediastinal low-grade fibromyxoid sarcoma with neck, axillary, and lung involvement Khamashta, Natalie Dalal, Ahmad Alashwas, Mo’men Idkedek, Mayar Abu-Akar, Firas Front Surg Surgery Low-grade fibromyxoid sarcoma is an extremely rare malignant neoplasm, with an incidence of 0.18 per million, and comprises 0.6% of all soft tissue sarcomas. It has a high recurrence rate and late metastatic spread and is chemotherapy and radiotherapy insensitive. This paper reports a case of an unusually large mediastinal low-grade fibromyxoid sarcoma in a 55-year-old patient. The tumor was engulfing the main blood vessels of the mediastinum, involving the lung, and extending beyond the chest cavity to involve the cervical and axillary regions. The patient has a 21-year history of frequent surgical resections for lesions that were repeatedly misdiagnosed as neurofibroma. The tumor was successfully resected by a challenging operation that involved mediastinal mass resection, chest wall mass resection, and wedge resection of the left upper lobe of the lung. The deceivingly benign-looking histology of this tumor makes it a commonly misdiagnosed one, requiring careful assessment by pathologists to reach the right diagnosis. Surgical resection with clear margins remains the treatment of choice for these lesions. Due to the behavior of this tumor, once detected and managed, extensive long-term follow-up is always recommended. Frontiers Media S.A. 2022-09-23 /pmc/articles/PMC9632998/ /pubmed/36338657 http://dx.doi.org/10.3389/fsurg.2022.988881 Text en © 2022 Khamashta, Dalal, Alashwas, Idkedek and Abu-Akar. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY) (https://creativecommons.org/licenses/by/4.0/) . 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 | Surgery Khamashta, Natalie Dalal, Ahmad Alashwas, Mo’men Idkedek, Mayar Abu-Akar, Firas Case report and review of literature: Resection of a huge mediastinal low-grade fibromyxoid sarcoma with neck, axillary, and lung involvement |
title | Case report and review of literature: Resection of a huge mediastinal low-grade fibromyxoid sarcoma with neck, axillary, and lung involvement |
title_full | Case report and review of literature: Resection of a huge mediastinal low-grade fibromyxoid sarcoma with neck, axillary, and lung involvement |
title_fullStr | Case report and review of literature: Resection of a huge mediastinal low-grade fibromyxoid sarcoma with neck, axillary, and lung involvement |
title_full_unstemmed | Case report and review of literature: Resection of a huge mediastinal low-grade fibromyxoid sarcoma with neck, axillary, and lung involvement |
title_short | Case report and review of literature: Resection of a huge mediastinal low-grade fibromyxoid sarcoma with neck, axillary, and lung involvement |
title_sort | case report and review of literature: resection of a huge mediastinal low-grade fibromyxoid sarcoma with neck, axillary, and lung involvement |
topic | Surgery |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9632998/ https://www.ncbi.nlm.nih.gov/pubmed/36338657 http://dx.doi.org/10.3389/fsurg.2022.988881 |
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