Myxofibrosarcoma of Right Buccal Mucosa with Rapid Nodal Recurrence After Radical Surgery: a Case Report

The World Health Organization (WHO) defines myxofibrosarcoma as the malignant fibroblastic neoplasm characterized by cellular pleomorphism, variably prominent myxoid stroma, and prominent elongated, thin-walled stromal blood vessels. Myxofibrosarcoma usually presents as a painless mass in the proximal extremities. Myxofibrosarcoma arising from head and neck region is extremely rare. We are reporting a rare case of myxofibrosarcoma of right buccal mucosa who developed rapid contralateral neck node recurrence after radical surgery. A 66-year-old female presented with 3 × 4-cm size ulcerative lesion at right buccal mucosa since 3 months. Biopsy from the ulcerative lesion was reported as poorly differentiated malignant epithelial neoplasm. Right buccal mucosa wide excision surgery along with right neck modified neck dissection was done. Histopathology and immunohistochemistry were suggestive of myxofibrosarcoma. After 4 weeks of surgery, she developed left level IB recurrent nodal metastasis. She received radical radiotherapy along with concurrent weekly cisplatin chemotherapy. After 3 months of radiotherapy, on first follow-up visit, no growth or residual/recurrent loco-regional disease was seen. Head-neck myxofibrosarcoma is an extremely rare tumor. Histopathological and immunohistochemistry investigations are essential in establishing the correct diagnosis of myxofibrosarcoma. Wide surgical excision with adjuvant radiotherapy is the primary treatment modality of myxofibrosarcoma. Role of prophylactic neck dissection and adjuvant chemotherapy is not well defined. Myxofibrosarcoma has high local recurrence rate. Our patient developed rapid nodal recurrence. Further research and studies are needed to explore the role of neck dissection, radiotherapy, and chemotherapy for myxofibrosarcoma of the head-neck region.