Endocrine Abstracts

Background: Undifferentiated pleomorphic sarcoma (UPS) of the thyroid is rare. It is often identified in its advanced stage, with distant metastasis, and has poor prognosis. This paper presents one of the less than 30 cases that have been documented ever since it was first identified in 1989. In the Philippines, there is no published report of a primary thyroid sarcoma.

Case Presentation: The author describes a 73-year-old female who presented with a rapidly enlarging neck mass, dysphagia, and weight loss. CT scan revealed a complex enhancing mass in the right thyroid gland measuring 16.5cm x 12.5cm with amorphous calcifications. She underwent tumor debulking, tube gastrostomy, tracheostomy, and radiation therapy. Biopsy revealed a malignant tumor exhibiting high-grade cellular features: storiform pattern and irregular fascicles with variable cellularity. She was discharged well after the initial admission, but succumbed two months after, exhibiting the dismal course and prognosis of this rare thyroid cancer.

Learning Points: There is still no consensus for the management of thyroid sarcoma but surgery plays a central role; this involves thyroidectomy plus excision of involved tissue with or without neck dissection. The recommendation for surgical margins of head and neck sarcomas is to do a complete tumor resection with as wide a margin as is feasible, with the least morbidity possible. Since surgical treatment alone gives poor results, adjuvant radiotherapy and/or chemotherapy is often utilized even if chemotherapy’s role for UPS remains unknown. The few existing studies on the advantages of chemotherapy detect no differences in overall survival.

Conclusion: Primary thyroid sarcoma is a rare malignancy that shares similar features with anaplastic thyroid cancer. The mainstay of treatment is surgery, radiotherapy, and clinical management of the malignancy’s complications; a multi-disciplinary team composed of surgical specialists and clinicians is crucial for the improved survival of these patients.