Thyroid angiosarcoma: A case report and review of literature.

Abstract

Thyroid angiosarcoma is a very aggressive and rare malignancy that is mostly seen in elderly of Alpine regions, related to high prevalence of iodine deficient goiter. The major diagnostic challenge is to distinguish between this entity and anaplastic carcinoma and to specify whether it is a primary or metastatic angiosarcoma. The objective of this work is to clarify this distinction through our case report and a revue of literature. We report the case of a 55 years-old female patient, with no medical history, admitted to the General Surgery Department of Ibn Sina Hospital in Rabat for the management of a cervical mass classified sonographically TI-RADS 6. It was also noted a lesion of the skin next to the thyroid lodge. The patient underwent total thyroidectomy with simple post-operative course. A third tumor location was discovered in the stomach during Oesophago-Gastro-Duodenoscopy (OGD). Microscopically, the thyroid, cutaneous and gastric tumors showed rows of oval to spindle-shaped cells, with marked cytonuclear atypia and high mitotic activity. The stroma was richly vascularized and fibro-inflammatory. Immunohistochemical studies demonstrated that the tumor cells showed positive staining for cytokeratin (AE1/AE3), CK7, ERG and CD31, while they exhibited no immunoreactivity for CK20, thyroglobulin or CD34. The first diagnostic challenge is to rule out anaplastic carcinoma. Using and the classification proposed by Cutlan et al. and a large immunohistochemical panel the diagnosis of epithelioid angiosarcoma was retained. Based on epidemiological and clinical criteria, the primary site in our case was more likely to be cutaneous. In summary, thyroid angiosarcoma is an uncommon thyroid neoplasm which poses a problem of differential diagnosis with anaplastic carcinoma and metastatic angiosarcoma. The use of a large panel of antibodies as well as the epidemiological and clinical data can solves the problem in the most difficult cases. • Thyroid angiosarcoma is a very rare malignancy that is mostly seen in elderly of Alpine regions, related to high prevalence of iodine deficient goiter. • It is a very aggressive tumor that can rapidly spread to the cervical lymph nodes, lungs, and brain or can metastasize to the duodenum, small bowel and large bowel. • Metastatic disease is associated with poor prognosis and limits the mean survival time to a few months after diagnosis and surgical treatment. • The first diagnostic challenge is to rule out an angiomatoid anaplastic carcinoma, using a large panel of antibodies. • The second challenge is to specify whether it is a primary or metastatic angiosarcoma especially when other sites are involved, based on clinical and epidemiological criteria.