翼突下顎部의 橫紋筋肉腫의 治驗例

A CASE REPORT OF RHABDOMYOSARCOMA OCCURRED IN THE PTERYGOMANDIBULAR REGION

Rhabdomyosarcorna, highly malignant tumor of the striated muscle, is the most common soft tissue sarcoma in the children. Most of the tumors were found within the first decade; 77% were 12 years or younger, half of them were under age 6. The usual sites of occurrence are the orbit, neck, and the nasopharynx. In the oral cavity the tumor is rarely originated, but frequently confused with a neuroblastoma or lymphosarcoma. The tumor presents initially as a painless growing, but followed by eventual involvement of lymphnodes & vital organs. The current treatment by combination of surgery, radiation & chemotherapy enhanced the survival rate markedly to about 70% from less than 10% of 1950 s. However the complicated sequelae make the prognosis worse. This is to report a case of rhabdomyosarcoma occurred in the pterygomandibular region of 3 year-old girl, treated with radiation and chemotherapy. The progress & follow-up results for 6 years were presented here with a brief review of literature. 1. Medial growing of the grayish-white mass of the pterygomandibular region supressed the maxillary arch development and the lateral expansion caused bowing of the mandibular ramus. 2. Incisional biopsy disclosed the tumor as a rhabdomyosarcoma, pleomorphic type. 3. Irradiation, 6000 Rad for 40 days resulted complete regression of tumor mass within 2 months, but leaving post-irradiation bum scar. 4. Multiple combination chemotherapy with Actinomycin D, Vincristine, and Cytoxan was rendered periodically for an year after the radiation. 5. Until now, 6 years later after above treatment, there is no sign of recurrence or metastasis. 6. Post-irradiation scarring and destruction of surrounding tissue developed to radionecrosis and complete resorption of mandibular ramus, causing facial disfigurement with an oro-facial fistula. 7. The through & through perforation of cheek, finger-breadth size, was closed by the tubed pedicle skin graft. 8. Further functional & esthetic reconstruction for the disfigured face and jaw will be recommended.