Navaratne L, Mathew R G, Kousparos G, McCombe A
The Royal London Hospital, London, UK.
Frimley Park Hospital, Camberley, UK.
Head Neck Pathol. 2017 Jun;11(2):139-145. doi: 10.1007/s12105-016-0745-2. Epub 2016 Jul 20.
Paragangliomas (PG) are very rare neuroendocrine tumours, arising from neural crest derived paraganglia of the autonomic nervous system. Primary thyroid paraganglioma (PTPG) is a rare site of PG and only 45 cases have been previously reported. The preoperative diagnosis of PTPGs presents a challenge as the clinical, cytological and histological features overlap with more common primary thyroid cancers. A 55 year old male was found to have significant enlargement of the left lobe of his thyroid. Following lobectomy, the thyroid lobe showed unencapsulated tumour which was positive for synaptophysin, CD56 and S100 (sustentacular cells). Post-operative imaging demonstrated incomplete resection. There was no post-operative radiotherapy and monitoring was by 6-12 monthly MRI. 48 months after his surgery he is alive and well with no evidence of disease progression. The diagnosis of PTPG was only made postoperatively, and although rare should be considered in the differential diagnosis of a hypervascular thyroid nodule.
副神经节瘤(PG)是非常罕见的神经内分泌肿瘤,起源于自主神经系统神经嵴衍生的副神经节。原发性甲状腺副神经节瘤(PTPG)是PG的罕见发病部位,此前仅报道过45例。PTPG的术前诊断具有挑战性,因为其临床、细胞学和组织学特征与更常见的原发性甲状腺癌重叠。一名55岁男性被发现甲状腺左叶明显肿大。甲状腺叶切除术后,甲状腺叶显示为无包膜肿瘤,突触素、CD56和S100(支持细胞)呈阳性。术后影像学检查显示切除不完全。未进行术后放疗,通过每6 - 12个月进行一次MRI监测。手术48个月后,他存活良好,无疾病进展迹象。PTPG的诊断仅在术后做出,尽管罕见,但在甲状腺高血运结节的鉴别诊断中应予以考虑。