Yepuri Natesh, Vanga Gautam R, Naous Rana, Kinthala Sudhakar
Department of Surgery, SUNY Upstate Medical University, Syracuse, NY 13210, USA.
Department of Pathology, SUNY Upstate Medical University, Syracuse, NY 13210, USA.
J Surg Case Rep. 2021 Apr 22;2021(4):rjab102. doi: 10.1093/jscr/rjab102. eCollection 2021 Apr.
Paragangliomas (PGs) are extremely rare multicentric neoplasms. Hereditary or familial PGs are associated with germline mutations in succinate dehydrogenase genes, seen in one-third of cases. Primary PGs of the thyroid are uncommon neuroendocrine neoplasms that account for 0.012% of all head and neck lesions. Although majority of these tumors are solitary, familial PGs are associated with synchronous tumors (carotid/vagal). We report an interesting case of primary thyroid PG in a patient with a previous history of a right carotid body, right vagal PGs and positive familial history, confining the differential diagnosis to recurrent lesions, which is the most common occurrence or new primary or a metastatic lesion. However, long interval and surgical anatomy suggests the diagnosis to be a primary lesion. In conclusion, although these lesions present multicentrically present at varying intervals, their occurrence at anatomically distinct sites should raise the concern for a new primary PG.
副神经节瘤（PGs）是极其罕见的多中心性肿瘤。遗传性或家族性PGs与琥珀酸脱氢酶基因的种系突变有关，三分之一的病例可见这种情况。甲状腺原发性PGs是罕见的神经内分泌肿瘤，占所有头颈部病变的0.012%。虽然这些肿瘤大多数是孤立性的，但家族性PGs与同步性肿瘤（颈动脉体/迷走神经）有关。我们报告了一例有趣的原发性甲状腺PG病例，患者既往有右颈动脉体、右迷走神经PGs病史且家族史呈阳性，鉴别诊断局限于复发病变，这是最常见的情况，或是新发原发性病变或转移性病变。然而，较长的间隔时间和手术解剖情况提示诊断为原发性病变。总之，虽然这些病变多中心出现且间隔时间不同，但它们在解剖学上不同部位的出现应引起对新发原发性PG的关注。
