Intramuscular low-grade fibromyxoid sarcoma: a case report
Kuo-Sheng Liao1, Wan-Ting Huang, Sheau-Fang Yang
1Department of Pathology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.
The Kaohsiung Journal of Medical Sciences
|July 17, 2009
Summary
Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor. Diagnosis is challenging due to its benign appearance, but molecular analysis, like the FUS-CREB3L2 fusion gene, aids identification.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Low-grade fibromyxoid sarcoma (LGFMS) is a rare, deep soft tissue neoplasm, often affecting young adult males.
- Histologically, LGFMS presents with bland spindle cells in fibrous and myxoid areas, mimicking benign lesions.
- Despite its appearance, LGFMS is known for local recurrence and late metastasis.
Observation:
- A case of LGFMS in the soleus muscle of a 29-year-old female is presented.
- Preoperative imaging suggested an intramuscular histiocytoma, highlighting diagnostic challenges.
- Immunohistochemical and ultrastructural findings supported fibroblastic differentiation.
Findings:
- LGFMS diagnosis is difficult due to bland histologic features.
- Immunohistochemistry can be limited in definitively diagnosing LGFMS.
- A characteristic t(7;16) translocation, creating the FUS-CREB3L2 fusion gene, is found in most LGFMS cases.
Implications:
- Cytogenetic and molecular analyses are crucial ancillary tools for accurate LGFMS diagnosis.
- Improved diagnostic accuracy can lead to better patient management and outcomes for this rare sarcoma.
- Understanding the genetic underpinnings of LGFMS enhances differential diagnosis in surgical pathology.
