Myxoid liposarcoma of thyroid in a 10-year-old child with Li-Fraumeni syndrome: A case report and review of literature.

Abstract: Myxoid liposarcoma of the thyroid is an extremely rare entity seen mostly in the retroperitoneum, extremities, and trunk; however, its occurrence in the head and neck region is reported to be less than 2%. Due to its rarity, evidence-based management is lacking. Here we report a case of a 10-year-old girl with Li-Fraumeni syndrome who presented with a large mass in the thyroid. She underwent hemi-thyroidectomy followed by six cycles of chemotherapy with Ifosfamide and Adriamycin after the histopathologically confirmed diagnosis of myxoid liposarcoma. Within a year, she developed recurrent disease in supraclavicular region and mediastinum, causing superior vena cava obstruction. For this, she received palliative radiotherapy followed by excision of the residual mass and second line chemotherapy. However, the disease progressed and the patient succumbed to the disease within 19 months of diagnosis. In view of the rarity of the condition, the literature does not recommend any standard approach. Surgical excision is a standard first-line approach. As it is difficult to achieve extensive surgical margins in the neck region due to the presence of critical structures in the vicinity, postoperative radiation may improve local control rates.