Thyroid Angiosarcoma-Rare Case or Hard to Find

Konstantinos Sapalidis; Nikos Kefes; Konstantinos Romanidis; Paul Zarogoulidis; Stelian Pantea; Otilia Constantina Rogoveanu; Ion Rogoveanu; Anastasios Vagionas; Katerina Zarampouka; Kosmas Tsakiridis; Isaac Kesisoglou

doi:10.12865/CHSJ.46.04.15

Thyroid Angiosarcoma-Rare Case or Hard to Find

Curr Health Sci J. 2020 Oct-Dec;46(4):433-437. doi: 10.12865/CHSJ.46.04.15. Epub 2020 Dec 31.

Affiliations

¹ 3rd Surgery Department, Medical School of Health Sciences, Aristotle University of Thessaloniki, "AHEPA" University Hospital, Thessaloniki, Greece.
² Second Department of Surgery, University Hospital of Alexandroupolis, Medical School, Democritus University of Thrace, Alexandroupolis, Greece.
³ Surgery Department, "Victor Babes" University of Medicine and Pharmacy Timisoara, 2nd Eftimie Murgu Sq., Timisoara 300041, Romania.
⁴ University of Medicine and Pharmacy of Craiova, Romania.
⁵ Oncology Department, General Hospital of Kavala, Greece.
⁶ Department of Pathology, Faculty of Medicine, Aristotle University of Thessaloniki, Thessaloniki, Greece.
⁷ Thoracic Surgery Department, "Interbalkan" European Medical Center, Thessaloniki, Greece.

Abstract

Introduction: Thyroid Angiosarcoma is a rare malignant condition of the thyroid gland with higher incidence reported in mountainous Alpine regions (Switzerland, Austria and northern Italy), featured with poor prognosis.

Case presentation: A 76 years old male patient presented in our hospital complaining about difficulty in breathing, altered voice (hoarseness), fatigue and dysphagia. Careful observation of the frontal neck region revealed hemorrhaging petechiae and purpura, while palpation indicated a large nodule movable with deglutition, presumably derived by the thyroid gland. Serum biochemical thyroid function tests were normal. Ultrasound of the thyroid gland showed enlargement of the right lobe with a nodular lesion. FNAC indicated a "suspicious for malignancy" lesion. After patient's consensus, total thyroidectomy took place accompanied by excision of infiltrated infrahyoid muscles. The cytopathologic results were positive for primary thyroid angiosarcoma. Patient's postoperative condition was regular, but he died of disease progression 6 months later.

Discussion: Angiosarcomas are malignant neoplasms arising from endothelial cells of blood vessels. They are most commonly found in skin, soft tissue, breast, bone, liver and spleen. Angiosarcomas tend to be highly hemorrhaging and invasive. Thyroid gland is a rare location of development. Cytopathologically they are grossly characterized by freely anastomosing vascular channels lined by atypical endothelial cells unusually enlarged, often multinucleated with many nucleoli and vacuoles into the cytoplasm containing fragments of erythrocytes. The immunohistological identity of angiosarcomas are endothelial line markers (CD31, CD34 and vimentin). Surgical excision when feasible is the first line treatment while adjuvant radio-and/or chemo-therapy are ambiguous. Infiltration of surrounding tissues and distant metastasis (lymph nodes and lungs) are negative prognostic factors.

Keywords: Thyroid cancer; angiosarcoma; surgery.

Publication types

Case Reports