Abstract
RATIONALE: Myxofibrosarcoma (MFS) is a subtype of soft tissue sarcoma that commonly occurs in the extremities but is rare in the neck. It is characterized by a high risk of local recurrence due to its specific infiltrative growth pattern. PATIENT CONCERNS: A 56-year-old male presented with a large mass in the right neck with disability of the right upper limb. DIAGNOSES: Magnetic resonance imaging revealed a 12.48 × 9.01 × 14.16 cm mass with a "tail sign." Resection with wide margins was performed to remove the tumor and the invaded surrounding tissues. Histopathological examination confirmed the diagnosis of high-grade MFS. INTERVENTIONS: The patient developed local recurrence 24 months after surgery and died of the disease 26 months after the recurrence. OUTCOMES: This case presents the largest MFS located in the neck reported to date. The treatment of giant MFS in the neck is challenging due to the proximity of vital structures including vessels, nerves, and other tissues. The invasive pattern of MFS facilitates extensive spread into surrounding tissues, complicating complete surgical removal. And insufficient margins frequently result in disease recurrence. LESSONS: This case highlights the significance of wide surgical margins in instances of extensive neck infiltration by MFS. However, further studies are required to confirm the specific margin values, and the advancement of effective adjuvant therapy is essential for improving patient survival.