| Popis: |
Synovial sarcoma is a rare malignant neoplasm of young adults with poor survival. These usually arise from large joints and articular tendons. We describe an unusual case of synovial sarcoma arising from the left retromolar trigone upon initial presentation. To the best of our knowledge, this case is the only patient with left retromolar trigone involvement recorded in the literature. A 33-year-old female presented to us with complaints of a lesion in the left retromolar trigone. An ulcerated lesion with irregular margins and a smooth base was noted at the left retromolar trigone. CT scan demonstrated a soft tissue mass predominantly involving the left buccal space measuring 5.0 × 4.2 × 2.8 cm superiorly extending into the left infratemporal region, closely abutting posterior lateral of the left maxillary sinus with gross bone thinning. Biopsy was taken from the temporal and infratemporal region, which was positive for synovial Sarcoma. She received 2 cycles of neoadjuvant chemotherapy AIM protocol followed by wide local excision, however, the margin status could not be ascertained. Subsequently, treatment with a total of 60 Gy adjuvant radiotherapy was given. Patient tolerated the treatment well and remained disease-free for 4.5 years. She then developed biopsy-proven recurrence at the primary site; her metastatic workup was negative. Unfortunately, the patient was lost to follow-up for 2 years and then presented with a very large lobulated mass in the left infratemporal fossa, now measuring 6.2 × 7.6 × 6.3 cm, encasing ramus of mandible, temporomandibular joint and muscle of mastication with intra-orbital extension. She received 2 cycles of chemotherapy with gemcitabine and docetaxel. However, she did not tolerate the chemotherapy well. Subsequently, she developed tumor bleeding and received palliative radiation for bleeding and pain control. Patient is now on the best supportive care, her general condition deteriorating rapidly with the passage of time. Synovial sarcoma of the retromolar trigone is a rare malignant tumor with a poor prognosis. As there is no standard treatment protocol for synovial sarcomas of the head and neck, optimal antineoplastic treatment remains unclear. Mainstay of treatment is surgical resection with high relapse rates. Radiation therapy, with or without chemotherapy, appears to improve survival. |
