Rapidly developing intrathoracic low‐grade fibromyxoid sarcoma: A case report
Abstract Low‐grade fibromyxoid sarcoma (LGFMS) is a rare mesenchymal tumor that primarily arises in the limbs and trunk of young adults, and rarely in the thoracic cavity. An 84‐year‐old Japanese woman presented with a right intrathoracic mass which was 8 cm in size. CT‐guided needle biopsy did not...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2023-08-01
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| Series: | Thoracic Cancer |
| Subjects: | |
| Online Access: | https://doi.org/10.1111/1759-7714.15020 |
| _version_ | 1797743889872846848 |
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| author | Eri Narukami Takashi Anayama Marino Yamamoto Yujiro Bunno Ryohei Miyazaki Hironobu Okada Mitsuko Iguchi |
| author_facet | Eri Narukami Takashi Anayama Marino Yamamoto Yujiro Bunno Ryohei Miyazaki Hironobu Okada Mitsuko Iguchi |
| author_sort | Eri Narukami |
| collection | DOAJ |
| description | Abstract Low‐grade fibromyxoid sarcoma (LGFMS) is a rare mesenchymal tumor that primarily arises in the limbs and trunk of young adults, and rarely in the thoracic cavity. An 84‐year‐old Japanese woman presented with a right intrathoracic mass which was 8 cm in size. CT‐guided needle biopsy did not provide a definitive diagnosis. Perioperatively, a mass was found in the right lower lobe of the lung and was suspected to have invaded the chest wall at the sixth–eighth ribs. A right lower lobectomy and combined chest wall resection were performed. Microscopic examination revealed that the tumor was a low‐grade spindle cell tumor originating from the pleura demonstrating focal invasion of the lung. The tumor exhibited positivity for MUC4, and FUS gene translocation was confirmed through fluorescence in situ hybridization. Unfortunately, 10 months postoperatively, tumor recurrence was noted as peritoneal dissemination, and the patient passed away 13 months postoperatively. Although LGFMS may be diagnosed histologically as a low‐grade tumor by needle biopsy, in this case, it was highly malignant. Postoperative long‐term regular medical follow‐up is recommended considering the highly malignant nature of the tumor and the high risk of local recurrence and pulmonary metastasis. |
| first_indexed | 2024-03-12T15:01:59Z |
| format | Article |
| id | doaj.art-ec383825c15e4b53927b73550337acbe |
| institution | Directory Open Access Journal |
| issn | 1759-7706 1759-7714 |
| language | English |
| last_indexed | 2024-03-12T15:01:59Z |
| publishDate | 2023-08-01 |
| publisher | Wiley |
| record_format | Article |
| series | Thoracic Cancer |
| spelling | doaj.art-ec383825c15e4b53927b73550337acbe2023-08-14T00:40:02ZengWileyThoracic Cancer1759-77061759-77142023-08-0114232314231910.1111/1759-7714.15020Rapidly developing intrathoracic low‐grade fibromyxoid sarcoma: A case reportEri Narukami0Takashi Anayama1Marino Yamamoto2Yujiro Bunno3Ryohei Miyazaki4Hironobu Okada5Mitsuko Iguchi6Department of Thoracic Surgery Chikamori Health Care Group Kochi JapanDepartment of Thoracic Surgery Chikamori Health Care Group Kochi JapanDepartment of Thoracic Surgery, Kochi Medical School Hospital Kochi University Nankoku JapanDepartment of Thoracic Surgery, Kochi Medical School Hospital Kochi University Nankoku JapanDepartment of Thoracic Surgery, Kochi Medical School Hospital Kochi University Nankoku JapanDepartment of Thoracic Surgery, Kochi Medical School Hospital Kochi University Nankoku JapanLaboratory of Diagnostic Pathology, Kochi Medical School Hospital Kochi University Nankoku JapanAbstract Low‐grade fibromyxoid sarcoma (LGFMS) is a rare mesenchymal tumor that primarily arises in the limbs and trunk of young adults, and rarely in the thoracic cavity. An 84‐year‐old Japanese woman presented with a right intrathoracic mass which was 8 cm in size. CT‐guided needle biopsy did not provide a definitive diagnosis. Perioperatively, a mass was found in the right lower lobe of the lung and was suspected to have invaded the chest wall at the sixth–eighth ribs. A right lower lobectomy and combined chest wall resection were performed. Microscopic examination revealed that the tumor was a low‐grade spindle cell tumor originating from the pleura demonstrating focal invasion of the lung. The tumor exhibited positivity for MUC4, and FUS gene translocation was confirmed through fluorescence in situ hybridization. Unfortunately, 10 months postoperatively, tumor recurrence was noted as peritoneal dissemination, and the patient passed away 13 months postoperatively. Although LGFMS may be diagnosed histologically as a low‐grade tumor by needle biopsy, in this case, it was highly malignant. Postoperative long‐term regular medical follow‐up is recommended considering the highly malignant nature of the tumor and the high risk of local recurrence and pulmonary metastasis.https://doi.org/10.1111/1759-7714.15020chest wall tumorFUS gene translocationLGFMSpulmonary invasion |
| spellingShingle | Eri Narukami Takashi Anayama Marino Yamamoto Yujiro Bunno Ryohei Miyazaki Hironobu Okada Mitsuko Iguchi Rapidly developing intrathoracic low‐grade fibromyxoid sarcoma: A case report Thoracic Cancer chest wall tumor FUS gene translocation LGFMS pulmonary invasion |
| title | Rapidly developing intrathoracic low‐grade fibromyxoid sarcoma: A case report |
| title_full | Rapidly developing intrathoracic low‐grade fibromyxoid sarcoma: A case report |
| title_fullStr | Rapidly developing intrathoracic low‐grade fibromyxoid sarcoma: A case report |
| title_full_unstemmed | Rapidly developing intrathoracic low‐grade fibromyxoid sarcoma: A case report |
| title_short | Rapidly developing intrathoracic low‐grade fibromyxoid sarcoma: A case report |
| title_sort | rapidly developing intrathoracic low grade fibromyxoid sarcoma a case report |
| topic | chest wall tumor FUS gene translocation LGFMS pulmonary invasion |
| url | https://doi.org/10.1111/1759-7714.15020 |
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