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Fibrosarcoma is a malignant mesenchymal tumor characterized by proliferation of fibroblasts producing collagen. Although it is now considered uncommon due to reclassification of many spindle cell sarcomas, fibrosarcoma remains a recognized entity in oral and maxillofacial pathology and exhibits aggressive local behavior with potential for recurrence and metastasis.
Definition and Classification
Fibrosarcoma is defined as a malignant tumor composed of spindle-shaped fibroblasts arranged in a characteristic herringbone pattern, with variable collagen production. It may be classified as:
- Infantile (congenital) fibrosarcoma
- Adult-type fibrosarcoma
In the head and neck region, most cases represent the adult type.
Epidemiology and Etiology
- Rare in the oral cavity, accounting for <1% of head and neck malignancies- Occurs across a wide age range; adult type commonly affects middle-aged adults
- No strong gender predilection
- Etiology is largely unknown
- Proposed associations include:
=Chronic irritation or trauma
=Malignant transformation of benign fibrous lesions (rare)
Common Sites of Occurrence
In the oral and maxillofacial region, fibrosarcoma may involve:
- Mandible and maxilla- Gingiva
- Buccal mucosa
- Tongue
- Soft tissues of the face and neck
Intraosseous lesions may present with bone destruction and tooth mobility.
Clinical Presentation
Clinical features depend on location and extent:
- Painless or painful rapidly enlarging mass- Firm, poorly circumscribed swelling
- Ulceration of overlying mucosa in advanced lesions
- Tooth displacement or loosening (in jaw lesions)
- Facial asymmetry
- Possible paresthesia in advanced disease
The clinical appearance may mimic benign reactive fibrous lesions in early stages.
Diagnostic Approach
Diagnosis requires exclusion of other spindle cell malignancies.
Key steps include:
- Radiographic imaging (CT/MRI) to assess soft tissue and bone involvement
- Incisional biopsy
- Histopathologic examination
- Immunohistochemical analysis to exclude other sarcomas
Immunohistochemistry
Fibrosarcoma typically shows:
- Vimentin positivity
- Negative staining for epithelial markers (cytokeratins)
- Negative for muscle markers (SMA, desmin)
- Negative for neural markers (S100)
Diagnosis is often one of exclusion.
Histopathological Features
Microscopic characteristics include:
- Interlacing fascicles of spindle-shaped cells
- Classic herringbone pattern
- Hyperchromatic, elongated nuclei
- Variable mitotic activity
- Collagen production between tumor cells
- Infiltrative growth into surrounding tissues
Tumor grading is based on cellularity, pleomorphism, and mitotic activity.
Differential Diagnosis
Important differential diagnoses include:
- Malignant peripheral nerve sheath tumor
- Leiomyosarcoma
- Spindle cell carcinoma
- Myofibrosarcoma
- Undifferentiated pleomorphic sarcoma
Accurate diagnosis requires correlation of morphology and immunoprofile.
Management and Treatment
Treatment is primarily surgical:
- Wide surgical excision with clear margins- Segmental resection for intraosseous tumors
- Adjuvant radiotherapy for high-grade or incompletely excised tumors
- Chemotherapy has limited and variable benefit
Reconstruction and functional rehabilitation are often necessary.
Prognosis
- Prognosis depends on tumor grade, size, and adequacy of surgical margins
- High local recurrence rates if excision is inadequate
- Hematogenous metastasis (lungs, bone) may occur
- Regional lymph node metastasis is uncommon
- Five-year survival varies widely (approximately 40–70%)
Role of Dental and Medical Professionals
Dental professionals play a key role in:
- Early detection of rapidly enlarging oral soft tissue masses
- Recognizing aggressive features in fibrous lesions
- Prompt biopsy and referral
- Long-term surveillance following treatment
For students, fibrosarcoma illustrates the importance of mesenchymal tumor pathology, pattern recognition, and exclusion diagnosis.
Conclusion
Fibrosarcoma is a rare but aggressive malignant fibroblastic tumor of the oral and maxillofacial region. Accurate diagnosis requires careful histopathologic evaluation and exclusion of other spindle cell malignancies. Early, wide surgical excision and long-term follow-up are essential to optimize patient outcomes.