Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literature
Abstract Background Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor characterized by bland histological features and aggressive clinical course. The most common anatomic locations of occurrence are the lower extremities, thorax, inguinal area, and upper limbs. Primary mediastinal sarcomas are...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2021-02-01
|
| Series: | Journal of Medical Case Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13256-020-02605-4 |
| _version_ | 1818969459627393024 |
|---|---|
| author | Mir Ibrahim Sajid Sidra Arshad Jamshid Abdul-Ghafar Saulat Hasnain Fatimi Nasir Ud Din |
| author_facet | Mir Ibrahim Sajid Sidra Arshad Jamshid Abdul-Ghafar Saulat Hasnain Fatimi Nasir Ud Din |
| author_sort | Mir Ibrahim Sajid |
| collection | DOAJ |
| description | Abstract Background Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor characterized by bland histological features and aggressive clinical course. The most common anatomic locations of occurrence are the lower extremities, thorax, inguinal area, and upper limbs. Primary mediastinal sarcomas are even rarer. To the best of our knowledge, only seven cases of primary mediastinal LGFMS have been reported in the literature. Here, we report a case of primary mediastinal LGFMS. Case presentation A 26-year-old Pakistani man presented with fever and vomiting for the past 2 months. On a routine chest x-ray, a mediastinal mass was incidentally found. Computed tomography (CT) scan showed a large circumscribed lobulated soft tissue density mass lesion in an anterior mediastinum. Grossly, the resected mass measured 17.0 × 12.0 × 11.0 cm. The cut surface was gray white with a whorled-like appearance and foci of calcification and cystic changes. Histologically, a spindle cell lesion was seen with alternating myxoid and hyalinized areas. The shaped cells were arranged in bundles. Immunohistochemical staining showed positive reactivity patterns with MUC4 and focally for epithelial membrane antigen (EMA). The diagnosis was confirmed as LGFMS. The patient is free of symptoms and recurrence 22 months after the surgery. Conclusion In conclusion, we report a rare case of primary mediastinal LGFMS in a young male patient that was discovered incidentally. Our patient is on regular follow-up to look for evidence of recurrence as these tumors are prone to recurrences. |
| first_indexed | 2024-12-20T14:20:55Z |
| format | Article |
| id | doaj.art-20bf1e3def3440819fd9d3879d36e4a9 |
| institution | Directory Open Access Journal |
| issn | 1752-1947 |
| language | English |
| last_indexed | 2024-12-20T14:20:55Z |
| publishDate | 2021-02-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Medical Case Reports |
| spelling | doaj.art-20bf1e3def3440819fd9d3879d36e4a92022-12-21T19:37:56ZengBMCJournal of Medical Case Reports1752-19472021-02-011511710.1186/s13256-020-02605-4Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literatureMir Ibrahim Sajid0Sidra Arshad1Jamshid Abdul-Ghafar2Saulat Hasnain Fatimi3Nasir Ud Din4Medical College, Aga Khan UniversityDepartment of Pathology and Laboratory Medicine, Aga Khan University HospitalDepartment of Pathology and Clinical Laboratory, French Medical Institute for Mothers and Children (FMIC)Section of Cardiothoracic Surgery, Department of Surgery, Aga Khan University HospitalDepartment of Pathology and Laboratory Medicine, Aga Khan University HospitalAbstract Background Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor characterized by bland histological features and aggressive clinical course. The most common anatomic locations of occurrence are the lower extremities, thorax, inguinal area, and upper limbs. Primary mediastinal sarcomas are even rarer. To the best of our knowledge, only seven cases of primary mediastinal LGFMS have been reported in the literature. Here, we report a case of primary mediastinal LGFMS. Case presentation A 26-year-old Pakistani man presented with fever and vomiting for the past 2 months. On a routine chest x-ray, a mediastinal mass was incidentally found. Computed tomography (CT) scan showed a large circumscribed lobulated soft tissue density mass lesion in an anterior mediastinum. Grossly, the resected mass measured 17.0 × 12.0 × 11.0 cm. The cut surface was gray white with a whorled-like appearance and foci of calcification and cystic changes. Histologically, a spindle cell lesion was seen with alternating myxoid and hyalinized areas. The shaped cells were arranged in bundles. Immunohistochemical staining showed positive reactivity patterns with MUC4 and focally for epithelial membrane antigen (EMA). The diagnosis was confirmed as LGFMS. The patient is free of symptoms and recurrence 22 months after the surgery. Conclusion In conclusion, we report a rare case of primary mediastinal LGFMS in a young male patient that was discovered incidentally. Our patient is on regular follow-up to look for evidence of recurrence as these tumors are prone to recurrences.https://doi.org/10.1186/s13256-020-02605-4Low-grade fibromyxoid sarcomaMediastinumAggressive behavior |
| spellingShingle | Mir Ibrahim Sajid Sidra Arshad Jamshid Abdul-Ghafar Saulat Hasnain Fatimi Nasir Ud Din Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literature Journal of Medical Case Reports Low-grade fibromyxoid sarcoma Mediastinum Aggressive behavior |
| title | Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literature |
| title_full | Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literature |
| title_fullStr | Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literature |
| title_full_unstemmed | Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literature |
| title_short | Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literature |
| title_sort | low grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass a case report and review of the literature |
| topic | Low-grade fibromyxoid sarcoma Mediastinum Aggressive behavior |
| url | https://doi.org/10.1186/s13256-020-02605-4 |
| work_keys_str_mv | AT miribrahimsajid lowgradefibromyxoidsarcomaincidentallydiscoveredasanasymptomaticmediastinalmassacasereportandreviewoftheliterature AT sidraarshad lowgradefibromyxoidsarcomaincidentallydiscoveredasanasymptomaticmediastinalmassacasereportandreviewoftheliterature AT jamshidabdulghafar lowgradefibromyxoidsarcomaincidentallydiscoveredasanasymptomaticmediastinalmassacasereportandreviewoftheliterature AT saulathasnainfatimi lowgradefibromyxoidsarcomaincidentallydiscoveredasanasymptomaticmediastinalmassacasereportandreviewoftheliterature AT nasiruddin lowgradefibromyxoidsarcomaincidentallydiscoveredasanasymptomaticmediastinalmassacasereportandreviewoftheliterature |