Patten Darren K, Flora Rashpal, Tolley Neil, Palazzo Fausto
Department of Biosurgery and Surgical Oncology, St Mary's Hospital, Imperial College Healthcare NHS Trust, London, UK.
Int J Surg Case Rep. 2012;3(2):92-6. doi: 10.1016/j.ijscr.2011.11.005. Epub 2011 Nov 22.
Medullary thyroid carcinoma (MTC) is an uncommon usually slowly progressing neuroendocrine tumour that arises from calcitonin (CT) producing parafollicular C cells of the thyroid gland. It accounts for approximately 5% of all thyroid cancers. The majority of MTCs are sporadic (75%) whilst 25% are part of the MEN 2 hereditary syndrome (MEN 2A and MEN 2B and familial MTC). Mutations of the proto-oncogene, RET (Rearranged during Transfection), found on chromosome 10q11, are present in more than 95% of hereditary MTCs and about 25% of sporadic MTCs. MTC metastasizes primarily via lymphatic spread, to central, and lateral nodal neck compartments and the anterior and superior mediastinum. Distant haematogenous spread targets the lungs, liver, bone and brain, and is presumed to be secondary to a lymphatic pathway. There are no previously documented reports of a focal pedunculated metastases located within the jugular vein. We present the first reported case of a metastatic MTC lesion found in the right internal jugular vein in a man with recurrent MTC.
甲状腺髓样癌（MTC）是一种罕见的、通常进展缓慢的神经内分泌肿瘤，起源于甲状腺产生降钙素（CT）的滤泡旁C细胞。它约占所有甲状腺癌的5%。大多数MTC是散发性的（75%），而25%是MEN 2遗传性综合征（MEN 2A、MEN 2B和家族性MTC）的一部分。原癌基因RET（转染期间重排）位于10q11染色体上，超过95%的遗传性MTC和约25%的散发性MTC存在该基因突变。MTC主要通过淋巴转移，转移至颈部中央和外侧淋巴结以及前纵隔和上纵隔。远处血行转移的靶器官为肺、肝、骨和脑，推测是继发于淋巴途径。此前没有关于颈静脉内局灶性带蒂转移瘤的文献报道。我们报告了首例在一名复发性MTC男性患者的右颈内静脉发现转移性MTC病变的病例。
