Authors
Nelo Joao Zeca Chihal Lima
Published in
Journal of the American Academy of Orthopaedic Surgeons. Global research & reviews. Volume 10. Issue 8. Aug 01, 2026. Epub Aug 06, 2026.
Abstract
Soft-tissue sarcomas (STSs) are a complex and remarkable heterogeneous group of uncommon malignancies originating from fat, muscle, and other connective tissues. They constitute approximately 1% of all cancers with 50 to 70 distinct histologic subtypes or tumor entities. This type of tumor, also known as "the loneliest cancer" because of its rarity, can manifest at any age, but they are most commonly diagnosed in individuals older than 40 years. They can rise from any anatomic location (somatic, visceral, or bone), but the extremity is the most common primary site. STS presents a notable clinical challenge due to its gradual onset and variable symptoms. Consequently, soft-tissue masses should be thoroughly evaluated to rule out malignancy. Some STSs can be aggressive and tend to have high recurrence and metastasis rates (40-50%), particularly in the lungs (80% of the cases) and less often in the liver, brain, bones, and peritoneum, where their occurrence might indicate a poor prognosis. The fact that they are rare and their histologic morphology varies and sometimes can overlap with different genetic, molecular, and clinical profiles contributes to their delayed and complex diagnoses. A high index of suspicion, coupled with appropriate clinical and radiologic assessment and biopsy, can help identify STS. A systematic, multidisciplinary team (e.g., orthopaedic surgeons, oncologists, radiologists, and pathologists) approach is essential for accurately diagnosing and treating soft-tissue sarcomas. Correlating clinical and radiologic information and involving a multidisciplinary team aid in accurate diagnosis. Biopsy, a crucial step, requires rigorous rules and appropriate technique selection. Histologic diagnosis can be challenging because of limited material and divergent interpretations. Advanced tools such as immunophenotypic panels and genetics can enhance accuracy. The fifth edition of the WHO manual can classify soft tissue, while the AJCC-TNM system can stage tumors. Surgical resection, often supplemented with radiation and chemotherapy, is the primary treatment for STS. However, some subtypes (e.g., liposarcoma, synovial sarcoma, and undifferentiated pleomorphic sarcoma) have high local recurrence rates or metastasis, leading to inconsistent treatment outcomes. Innovative immune and radiation therapy treatment regimens and targeted therapy can offer potential effective alternatives for these challenging cases.
PMID:
42562371
Bibliographic data and abstract were imported from PubMed on 07 Aug 2026.
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