Yu Juan, Steiner Federico A, Muench Jeffrey P, Gourgiotis Loukas, Skarulis Monica C, Altemus Rosemary M, Libutti Steven K, Merino Maria J, Sarlis Nicholas J
Division of Intramural Research, National Institute of Child Health and Human Development, Bethesda, Maryland, USA.
Thyroid. 2002 May;12(5):427-32. doi: 10.1089/105072502760043521.
Angiosarcoma is a malignant growth of endothelial origin, uncommon in the head and neck. We present the case of a 38-year-old woman with long-standing goiter who presented with a rapidly growing 6.0-cm neck mass. Fine-needle aspiration biopsy of the tumor showed features of "undifferentiated thyroid carcinoma (ThyrCa)." Total thyroidectomy resulted in extirpation of all gross disease. Pathology revealed a high-grade angiosarcoma of the neck invading the thyroid gland, coexisting with papillary ThyrCa (follicular variant) in the contralateral lobe. Aggressive external electron beam radiotherapy was initiated for local control. Despite the absence of systemic dissemination initially, bulky neck recurrences, and pulmonary metastases developed rapidly, leading to the patient's demise on postoperative day 41. Autopsy showed metastatic disease involving most organs. This case illustrates that neck angiosarcomas need to be considered in the differential diagnosis of "poorly differentiated" thyroid malignancies. These soft tissue neck tumors may complicate postoperative management due to their bleeding tendency and aggressive infiltrative behavior, and carry a dismal prognosis because of the rapidity of development of local recurrence and distant metastases.
血管肉瘤是一种起源于内皮细胞的恶性肿瘤,在头颈部并不常见。我们报告一例38岁患有长期甲状腺肿的女性患者,其颈部出现一个迅速增大的6.0厘米肿块。对该肿瘤进行细针穿刺活检显示为“未分化甲状腺癌(ThyrCa)”特征。全甲状腺切除术切除了所有肉眼可见的病灶。病理检查显示为颈部高级别血管肉瘤侵犯甲状腺,对侧叶并存乳头状ThyrCa(滤泡变体)。开始进行积极的外照射电子束放疗以控制局部病变。尽管最初没有全身播散,但颈部大块复发和肺转移迅速出现,导致患者在术后第41天死亡。尸检显示转移病灶累及大多数器官。该病例表明,在“低分化”甲状腺恶性肿瘤的鉴别诊断中需要考虑颈部血管肉瘤。这些颈部软组织肿瘤因其出血倾向和侵袭性浸润行为可能使术后管理复杂化,并且由于局部复发和远处转移发展迅速,预后不佳。