Secretory Carcinoma of the Thyroid in a 49-Year-Old Man Treated with Larotrectinib: Protracted Clinical Course of Disease Despite the High-Grade Histologic Features.

IF 4.1
Head and neck pathology Pub Date : 2022-06-01 Epub Date: 2021-10-16 DOI:10.1007/s12105-021-01386-6
Maelle Saliba, Abhinita S Mohanty, Alan L Ho, Alexander Drilon, Snjezana Dogan
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引用次数: 5

Abstract

Secretory carcinoma of the thyroid gland is histologically and genetically similar to its mammary and salivary gland counterparts. Unlike differentiated thyroid carcinomas of follicular cell origin, thyroid SC is not a thyroglobulin-producing tumor and would not be amenable to radioactive iodine therapy. Instead, these carcinomas may respond to targeted therapy with TRK inhibitors, which further emphasizes the importance of their recognition among morphologically similar thyroid entities. Based on eleven cases reported to date, most primary thyroid SC tend to present as locally advanced malignancies and are characterized by frequent recurrences and long-term survival. High-grade histologic features, increased mitotic count and necrosis have been described but their impact on clinical course and outcome remains unclear. We hereby report the case of a primary SC with high-grade features arising in the thyroid of a 49-year-old man, who was treated with Larotrectinib for his second recurrence. The patient achieved a durable response that lasted for 18 months but then he continued to progress and died of disease 181 months after the diagnosis.

Abstract Image

Abstract Image

49岁男性甲状腺分泌性癌的larorectinib治疗:尽管有高级别的组织学特征,但临床病程延长。
甲状腺的分泌性癌在组织学和遗传学上与乳腺和唾液腺癌相似。与源自滤泡细胞的分化型甲状腺癌不同,甲状腺SC不是一种产生甲状腺球蛋白的肿瘤,也不适合放射性碘治疗。相反,这些癌症可能对TRK抑制剂的靶向治疗有反应,这进一步强调了在形态相似的甲状腺实体中识别它们的重要性。根据迄今为止报道的11例病例,大多数原发性甲状腺SC倾向于表现为局部晚期恶性肿瘤,其特点是经常复发和长期生存。高级别组织学特征,有丝分裂计数增加和坏死已被描述,但其对临床过程和结果的影响尚不清楚。我们在此报告一例原发性SC与高级别特征出现在甲状腺的49岁男性,谁是用larorectinib治疗他的第二次复发。患者获得了持续18个月的持久反应,但随后病情继续恶化,并在诊断181个月后死于疾病。
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