Rapidly developing intrathoracic low-grade fibromyxoid sarcoma: A case report
Eri Narukami1,2, Takashi Anayama1,2, Marino Yamamoto2
1Department of Thoracic Surgery, Chikamori Health Care Group, Kochi, Japan.
Thoracic Cancer
|July 4, 2023
Summary
Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor. Despite initial low-grade diagnosis, this intrathoracic LGFMS case showed high malignancy with rapid recurrence and metastasis.
Area of Science:
- Oncology
- Pathology
Background:
- Low-grade fibromyxoid sarcoma (LGFMS) is a rare mesenchymal neoplasm typically affecting young adults in the limbs and trunk.
- Intrathoracic LGFMS is exceptionally rare, posing diagnostic and therapeutic challenges.
Observation:
- An 84-year-old woman presented with a large right intrathoracic mass initially undiagnosed by CT-guided biopsy.
- Surgical resection revealed a pleural-based, low-grade spindle cell tumor with focal lung invasion and chest wall involvement.
Findings:
- Histopathological analysis confirmed LGFMS, positive for MUC4, with FUS gene translocation identified.
- The tumor demonstrated aggressive behavior, recurring as peritoneal dissemination 10 months post-surgery, leading to death within 13 months.
Implications:
- This case highlights that LGFMS, even with initial low-grade histological findings, can exhibit high malignancy and aggressive behavior.
- Long-term, regular medical follow-up is crucial for patients with LGFMS due to the high risk of recurrence and metastasis.
