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Fibrosarcoma originating in the mandible
Andresa Borges Soares1, Lauro Henrique Sousa Lins, Adriana Parizzoto Macedo
1Department of Oral Diagnosis, Oral Pathology Section, Dental School of Piracicaba, State University of Campinas, Piracicaba, Brasil.
Medicina Oral, Patologia Oral Y Cirugia Bucal
|May 2, 2006
Summary
This case study details a rare high-grade intra-osseous fibrosarcoma in a teenager's mandible. Despite aggressive treatment including surgery, radiation, and chemotherapy, the patient developed metastases and ultimately succumbed to the disease.
Area of Science:
- Oncology
- Oral Pathology
- Surgical Pathology
Background:
- Fibrosarcoma, a rare malignant mesenchymal neoplasm of fibroblasts, infrequently occurs in the oral cavity.
- Intra-osseous fibrosarcoma of the mandible presents a diagnostic and therapeutic challenge due to its rarity and potential for aggressive behavior.
Observation:
- A case of primary fibrosarcoma in the mandible of a 16-year-old girl is presented.
- Microscopic examination revealed a high-grade tumor with spindle-shaped cells, increased mitotic activity, and nuclear pleomorphism.
- Immunohistochemical analysis showed vimentin positivity and negativity for markers including S-100, cytokeratins, and CD34.
Findings:
- The final diagnosis was high-grade intra-osseous fibrosarcoma based on integrated clinical, radiological, histological, and immunohistochemical findings.
- The patient underwent radical surgery with mandibular reconstruction.
- Despite initial treatment, the patient developed multiple metastases within 21 months, indicating an aggressive tumor phenotype.
Implications:
- This case underscores the aggressive nature of high-grade intra-osseous fibrosarcoma, even in young patients.
- The limited immunohistochemical profile highlights the importance of a comprehensive diagnostic approach.
- Aggressive adjuvant therapies, including radiation and chemotherapy, were employed but ultimately unsuccessful, emphasizing the need for further research into effective treatments for this rare malignancy.