Thyroid angiosarcoma is an uncommon thyroid carcinoma and its incidence is the highest in the European Alpine regions. Thyroid angiosarcoma is also a very aggressive tumor that can rapidly spread to the cervical lymph nodes, lungs, and brain or can metastasize to the duodenum, small boewl, and large bowel. Although it is histologically well defined, clear-cut separation between the angiosarcoma and anaplastic thyroid carcinoma is difficult. A 49-year-old Caucasian female patient, born and resident in Southern Italy (Calabria), in an iodine-sufficient area, was admitted to the Surgery Department because she presented with a painless mass in the anterior region of neck enlarged rapidly in the last three months. After total thyroidectomy and right cervical lymphadenectomy, postoperative histological examination revealed the presence of a thyroid angiosarcoma with positive staining for CD31 and for both Factor VIII-related antigen and Vimentin and only partially positive for staining pancytokeratin and presence of metastasis in cervical, supraclavicular, mediastinal and paratracheal lymph nodes. The patient started adjuvant chemotherapy and she was treated for 6 cycles with Doxorubicin, Dacarbazine, Ifosfamide, and Mesna (MAID). After 22 months from surgery, the patient is still alive without both local and systemic recurrence of the disease.
Thyroid angiosarcoma is a very rare disease. As previously reported by other authors, its incidence is the highest in the European Alpine regions and in some European areas as Northern Italy, Austria, and Switzerland it can constitute the 2–10% of malignant thyroid tumors [
Although it is histologically defined as cleft-like anastomosing spaces lined by large, atypical cells of endothelial lineage [
Angiosarcoma treatment is complex and consisting of surgery, chemotherapy, and radiotherapy.
We report the first case of thyroid angiosarcoma identified in Southern Italy (Calabria), in nonalpine area.
On November 2011, a 49-year-old Caucasian female patient, born and resident in Southern Italy (Calabria), in an iodine-sufficient area, was admitted to the Surgery Department because she presented with a painless mass in the anterior region of the neck. In her history, longstanding nodular goiter with regressive changes including intranodular hemorrhage was reported; the goiter had been noticed ten years ago and the patient was treated with levothyroxine. However, the neck mass enlarged rapidly in the last three months. Clinical examination of the thyroid gland showed a remarkable enlargement of the right lobe in which a nodular lesion was present. In contrast, the left lobe of the thyroid gland was nonpalpable. There was evidence of involvement of cervical lymph nodes.
Thyroid gland high-resolution ultrasonography confirmed the enlargement only of the right lobe that showed a morphology suggestive of an autoimmune thyroid disease and the presence of nodular lesion measuring
The patient showed also iron deficiency anemia. Indeed, the blood values were the following: red blood cell count (RBC) is
Neck computerized tomography confirmed the presence of a large vascular mass that measured
After iron supplementation and normalization of the hemoglobin value, the patient underwent total thyroidectomy and right cervical lymphadenectomy.
Histological examination showed a well-circumscribed nodule confined within the thyroid and with large areas of necrotic fibrinous tissue. Microscopic examination disclosed a proliferation of epithelioid cells with enlarged pleomorphic nuclei and prominent nucleoli (Figure
(a) Proliferation of cellular elements with epithelial appearance, with large vesicular nucleus, and with prominent nucleolus. (b) Areas consist of irregular spaces containing material blood bordered by atypical cells with intraluminal papillary projections.
On immunohistochemical examination, tumor cells showed both extensive and intensive immunoreactivity for CD31 (Figure
(a) Angiosarcoma cells show positivity for CD31 antigen (monoclonal mouse clone JC70A—Dako—40x). (b) Factor VIII-related antigen positivity in the angiosarcoma cells (policlonal rabbit—DAKO—40x). (c) Tumor cells express partially pancytokeratin (CK-) related antigen (cytokeratin monoclonal mouse clone AE1/AE3—DAKO—40x).
Total body computerized tomography performed after surgical treatment revealed the presence of right cervical, supraclavicular, mediastinal, and paratracheal lymphadenopathy. There were no alterations like metastatic lesions in lung, liver, spleen, and/or other organs.
One month after the total thyroidectomy, the patient started adjuvant chemotherapy and she was treated for 6 cycles with Doxorubicin, Dacarbazine, Ifosfamide, and Mesna (MAID).
On June 2012, December 2012 and June 2013, after adjuvant chemotherapy, a total body TAC has been performed and there were no alterations like metastatic lesions in lymph nodes, lung, liver, spleen, and/or other organs. The patient is still alive without both local systemic recurrence of the disease after 22 months from surgery.
In this paper we describe the first case of thyroid angiosarcoma identified in Southern Italy (Calabria), in nonalpine area. The incidence of this uncommon tumor is highest in European Alpine regions [
Surgical resection to obtain the local control of thyroid angiosarcoma remains the still point of primary treatment. However, thyroid angiosarcoma is a very aggressive tumor. Indeed, it can rapidly spread to the cervical lymph nodes, lungs, and brain or can metastasize to the duodenum, small bowel and large bowel and induce severe bleeding [
In conclusion, thyroid angiosarcoma is an uncommon thyroid carcinoma and it is also a very aggressive tumor with a poor prognosis. Surgical resection remains the still point of primary treatment. However, our case showed that MAID combination chemotherapy may have an important role in the treatment of thyroid angiosarcoma in nonolder patients.
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The authors declare that there is no conflict of interests regarding the publication of this paper.
Nadia Innaro was the patient's physician; Nadia Innaro, Elena Succurro, and Franco Arturi had full access to all of the data in the study and took responsibility for the integrity of the data and the accuracy of the data analysis; Giuseppe Tomaino, Elena Succurro, and Franco Arturi were responsible for both acquisition and, literature review; Elena Succurro and Franco Arturi drafted the paper. All authors have read the paper and agreed on the content. Nadia Innaro and Elena Succurro contributed equally to this work.
The authors wish to thank Dr. V. Tucci “Pugliese Hospital” for his contribution in the histological diagnosis.