Non-alpine primary thyroid angiosarcoma.

Area of Science:

• Oncology
• Pathology
• Endocrinology

Background:

• Thyroid angiosarcoma is an exceptionally rare malignancy, often linked to iodine deficiency and endemic goiter in specific regions.
• It is characterized by its aggressive nature and typically poor patient prognosis.

Observation:

• A case report of a 49-year-old Portuguese female with a rapidly growing anterior neck mass and dysphonia.
• Initial investigations revealed a large, heterogeneous thyroid nodule; fine needle aspiration was inconclusive, necessitating a biopsy.
• Histopathology suggested a mesenchymal tumor with vascular features, positive for endothelial markers and negative for thyroid-specific markers.

Findings:

• Pre-operative imaging identified multiple pulmonary nodules, indicative of metastatic disease at the time of diagnosis.
• Histologic examination confirmed thyroid angiosarcoma following total thyroidectomy and lymph node dissection.
• Despite palliative chemotherapy, the patient experienced disease progression and succumbed to respiratory failure 29 months post-diagnosis.

Implications:

• This case underscores the aggressive behavior of thyroid angiosarcoma, particularly when presenting with pulmonary metastases.
• The rarity of this condition limits the available data on optimal management and treatment strategies.
• Further research is needed to improve understanding and therapeutic approaches for thyroid angiosarcoma.