A Case Report of Surgical and Postoperative Treatment for Thyroid Angiosarcoma with Pulmonary Metastasis

Thyroid angiosarcoma (TAS) is an extremely rare and highly aggressive cancer, representing less than 1% of all sarcomas. Typically diagnosed in individuals aged 50-80, with a higher incidence in women, TAS has a poor prognosis due to its tendency to metastasize, leading to a low 5-year survival rate. Due to its rarity, standardized treatment approaches are lacking, often involving a combination of surgery, chemotherapy, and radiation. This report presents TAS in a Japanese male.

A 78-year-old male with pre-existing conditions presented with a 30-year history of a slow-growing thyroid tumor that exhibited rapid enlargement 4 months prior to surgery. Initial fine-needle aspiration cytology was no malignancy. Examinations revealed a firm, poorly mobile 10 cm mass in the anterior neck, and left pleural effusion. Notably, papules developed at the aspiration site and progressively enlarged. Preoperative imaging indicated a malignant thyroid tumor, prompting a right thyroid lobectomy with resection of anterior neck muscles and overlying skin. Histological analysis confirmed a highly hemorrhagic angiosarcoma with infiltration into surrounding tissues. Immunohistochemical findings supported the diagnosis of TAS. A comprehensive genomic profiling testing yielded no specific therapeutic recommendations. Paclitaxel therapy was initiated 2 months after surgery, resulting in the shrinking of pulmonary nodules and the decreasing left pleural effusion. The patient subsequently died from septic shock due to a urinary tract infection 5 months after starting chemotherapy, without evidence of cervical recurrence or neutropenia during treatment.

This case highlights the perioperative management of a rare primary TAS. In the absence of established treatment guidelines, surgical resection followed by paclitaxel administration could be a potential therapeutic strategy to control disease progression. An accumulation of case reports is needed to better understand this aggressive malignancy and to facilitate the development of optimized therapeutic strategies.