Abstract
Background/Objectives: Low-grade myofibroblastic sarcoma is a rare malignant myofibroblastic tumor, first described by Gabbiani et al. in 1971. It predominantly affects adult males and is uncommon in children. The tumor can occur anywhere in the body, with a preference for the limbs and head and neck regions, including the oral cavity. Methods: A case report of a 22-year-old male patient with no systemic conditions who presented with a painless, red, firm swelling between teeth 34 and 35 was examined. Panoramic radiography was unremarkable. The lesion measured 19 × 8 mm. Histological analysis revealed a spindle cell proliferation with atypia. Immunohistochemical staining was positive for SMA and showed a low Ki-67 proliferative index, while negative for Desmin, CD34, and beta-catenin. The final diagnosis was low-grade myofibroblastic sarcoma. Results: After surgical excision, the lesion recurred within one month. The patient was referred to the Portuguese Institute of Oncology for further treatment. Conclusions: Low-grade myofibroblastic sarcoma is a rare tumor with a tendency for local recurrence and metastasis. Accurate diagnosis, which includes histological examination and immunohistochemistry, is essential to differentiate it from other benign and malignant lesions. This case highlights the importance of an incisional biopsy to confirm diagnosis prior to surgical excision, thereby reducing the risk of relapse and enabling appropriate treatment planning.
Published Version
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