Solitary fibrous tumor: molecular hallmarks and treatment for a rare sarcoma.
Loading...
Embargo End Date
ICR Authors
Authors
Smrke, A
Thway, K
H Huang, P
Jones, RL
Hayes, AJ
Thway, K
H Huang, P
Jones, RL
Hayes, AJ
Document Type
Journal Article
Date
2021-09-01
Date Accepted
2021-04-30
Abstract
Solitary fibrous tumor (SFT) is a rare soft tissue sarcoma subtype which mainly affects adults in the fifth and sixth decades of life. Originally part of a spectrum of tumors called hemangiopericytomas, classification has been refined such that SFTs now represent a distinct subtype. The identification of NAB2-STAT6 fusion in virtually all SFTs has further aided to define this rare subgroup. SFTs have a spectrum of behavior from benign to malignant, with evidence suggesting risk of metastases related to age at diagnosis, extent of necrosis, mitotic rate and tumor size. The standard treatment for localized disease is surgical excision with or without radiotherapy. Retrospective and prospective evidence suggests antiangiogenic treatment is effective for unresectable disease. Further translational work is required to understand the biology driving the differential behavior and identify more effective treatments for patients with metastatic disease.
Citation
Future oncology (London, England), 2021, 17 (27), pp. 3627 - 3636
Source Title
Publisher
TAYLOR & FRANCIS LTD
ISSN
1479-6694
eISSN
1744-8301
Research Team
Sarcoma Clinical Trials (R Jones)
Molecular and Systems Oncology
Sarcoma and Melanoma Surgery
Molecular and Systems Oncology
Sarcoma and Melanoma Surgery