An extremely rare case of thyroid malignancy from the non-Alpine region: Angiosarcoma

Int J Surg Case Rep. 2016:19:92-6. doi: 10.1016/j.ijscr.2015.12.028. Epub 2015 Dec 23.

Abstract

Introduction: Thyroid angiosarcoma is a rather rare malignancy featuring a poor prognosis, and which may interfere with other aggressive thyroid tumors; it is usually seen in the Alpine region.

Case presentation: A 74-year-old male was referred to our center with complaints of progressive neck swelling and dyspnea. He had multiple nodules featuring cystic degeneration and calcifications in the thyroid gland, together with multiple lymphadenopathies of the neck region. Fine-needle aspiration cytology (FNAC) confirmed the presence of anaplastic carcinoma. A total thyroidectomy was performed. During the postoperative period, multiple drainage were performed for recurrent hematomas, but hematoma development could not be prevented. On postoperative day 7, the patient died due to multiple-system failure. Histopathological investigation of the thyroidectomy specimen indicated that the lesion was an angiosarcoma.

Discussion: The cytological diagnosis of thyroid angiosarcoma is quite difficult. Extracapsular invasion and distant organ metastasis during surgery are known as strong and negative prognostic factors for thyroid angiosarcoma. Treatment is quite difficult, since this tumor is locally aggressive, destructive, and features a high recurrence rate. In this case, since extracapsular invasion, as well as lymph node and lung metastasis were present at the time of surgery; the expected survival time was quite short.

Conclusion: This case shows that during differential diagnosis, patients initially diagnosed with anaplastic carcinoma via FNAC may actually present with angiosarcoma. It may be helpful to review the treatment modalities for this cancer type, which has a rather poor prognosis and features severe bleeding, as well as local and distant metastasis.

Keywords: Head and neck malignancy; Hematoma; Primary angiosarcoma; Surgery; Thyroid gland.