Kondapalli Ananya, Redd Lucas, DeBlanche Lorraine, Oo Yin
Department of Medicine, Howard Hughes Medical Institute, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
VA North Texas Health Care System, Dallas, Texas, USA.
BMJ Case Rep. 2019 Jun 26;12(6):e228862. doi: 10.1136/bcr-2018-228862.
Mesenchymal origin of primary thyroid angiosarcomas (TAS) is extremely rare and comprises less than 1% of primary thyroid cancer worldwide. While TAS are most commonly occurring in the Alpine region, there are multiple reported cases of TAS in non-Alpine regions. Diagnosis of TAS is commonly made after thyroidectomy as cytologic diagnosis can be challenging due to paucity of cells, presence of necrosis and unawareness of the disease due to rarity. We report a case of primary TAS diagnosed by cytology in a 56-year-old man who presented with a sudden onset of left neck pain, swelling and haemoptysis. He was later noted to have suspicious nodules on both lobes of thyroid on ultrasound. Fine needle aspiration of thyroid nodules showed malignant epithelioid cells. The diagnosis of TAS was made based on positive endothelial markers such as thrombomodulin and CD31, with many pertinent negatives, including negative cytokeratins,thyroid transcription factor (TTF1), thyroglobulin, calcitonin and carcinoembryonic antigen (CEA).
原发性甲状腺血管肉瘤(TAS)起源于间充质极为罕见,在全球原发性甲状腺癌中所占比例不到1%。虽然TAS最常发生在阿尔卑斯地区,但非阿尔卑斯地区也有多个TAS病例报告。TAS的诊断通常在甲状腺切除术后进行,因为由于细胞数量少、存在坏死以及由于该病罕见而不为人知,细胞学诊断可能具有挑战性。我们报告一例56岁男性通过细胞学诊断的原发性TAS病例,该患者突然出现左颈部疼痛、肿胀和咯血。后来经超声检查发现其甲状腺两叶均有可疑结节。甲状腺结节细针穿刺显示恶性上皮样细胞。基于血栓调节蛋白和CD31等阳性内皮标志物,同时排除了许多相关阴性指标,包括细胞角蛋白、甲状腺转录因子(TTF1)、甲状腺球蛋白、降钙素和癌胚抗原(CEA)阴性,从而诊断为TAS。