Rationale: Undifferentiated pleomorphic sarcoma is extremely rare in the head and neck area, and the aggressive nature of this tumor has not been previously described.
Patient concerns: Therefore, we report a unique case of a very rapidly growing primary undifferentiated pleomorphic sarcoma in the eyelid. A 67-year-old woman presented with a left lower lid mass. She had no previous history of surgery, skin malignancy, or radiation to the eyelid. On initial presentation, the patient showed a 2 × 1 × 1 cm firm, yellow nodule in the left lower eyelid.
Diagnoses: We planned complete surgical removal of the mass; however, the patient was lost to follow-up. One month after the initial visit, the mass had markedly enlarged to 3 × 2.2 × 2.3 cm and a new 2 × 2 × 2 cm mass was observed in the left lower eyelid.
Interventions: Complete surgical resection of the tumor was performed with a myocutaneous free flap. At that time, two masses were emerged, which had grown to 8 × 8 × 5 cm.
Outcomes: The patient died from sepsis caused by pneumonia 2 months after surgery without evidence of local recurrence.
Lessons: Primary undifferentiated pleomorphic sarcoma in the eyelid is extremely rare. Surgeons should be aware of this abruptly presenting, rapidly growing primary eyelid tumor and it is essential to excise the tumor completely as soon as possible.