Background

Fibrosarcomas are malignant tumours made of fibroblasts with variable collagen synthesis and a “herringbone” structure. Previously, the reported incidence was much higher because many non-mesenchymal and mesenchymal tumors which mimic fibrosarcomas were incorrectly diagnosed as fibrosarcoma.

In recent decades this has changed, so the incidence rate has been dropping steadily. There are two forms of fibrosarcomas: infantile or congenital fibrosarcomas, and adult-type fibrosarcomas. Adult-type fibrosarcomas are extremely malignant, whereas infantile fibrosarcomas rarely metastasise.

Epidemiology

Fibrosarcomas are more prevalent in middle-aged and elderly individuals, and they are extremely uncommon in youngsters. Males are slightly more likely to be affected. Some common sites which develop fibrosarcoma include deep soft tissues in the head, neck, trunk, and within the pelvic region.

In an analysis of adult fibrosarcomas, 80% were categorized within grades 2 and 3 (high-grade). Out of the lesions which were diagnosed as grade 1, 25% later progressed into high-grade sarcomas over time.

Anatomy

Pathophysiology

Fibrosarcoma can develop from mesenchymal cells and is formed of uncontrollably proliferating spindle-shaped fibroblasts. Most often, fibrosarcoma can arise in the tendons and fascia of deep tissues, although it can also arise in the medullary canal or periosteum of bones. Additionally, preexisting bone lesions or irradiated tissues may cause subsequent bone fibrosarcoma.

Etiology

Fibrosarcoma often arises from fascia and tendons of deep tissue, although they can also develop in the medullary canal or periosteum of bones as a primary or secondary tumor. Bone fibrosarcoma can be caused by bone injuries which occurred due to radiation therapy or physical trauma.

Genetics

Prognostic Factors

The 2-year survival rate for fibrosarcoma is below 70%, and the 5-year survival rate is below 55%.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Future Trends

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References

https://www.ncbi.nlm.nih.gov/books/NBK560759/