Department of Ultrasound, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
Orphanet J Rare Dis. 2024 Mar 26;19(1):136. doi: 10.1186/s13023-023-02867-3.
Diffuse sclerosing variant of papillary thyroid carcinoma (DSVPTC) is a rare but high invasive subtype of papillary thyroid carcinoma, which mandates an aggressive clinical strategy. Few studies have focused on the sonographic characteristics of DSVPTC and the role of ultrasound in diagnosis and treatment of this variant remains unknown. This study aimed to identify and understand DSVPTC more accurately under ultrasound in correlation with pathology.
The ultrasound characteristics and histopathologic sections of 10 lesions in 10 DSVPTC patients who underwent thyroid surgery at our center between 2014 and 2020 were reviewed and compared with 184 lesions in 168 classic variant of papillary thyroid carcinoma (cPTC) patients.
6 DSVPTC cases (60%) showed the "snowstorm" pattern on sonogram and 4 cases (40%) presented hypoechoic solid nodules only. Vague borders (100.0% vs. 18.5%, P = 0.019) and abundant microcalcifications (66.7% vs. 10.9%, P = 0.037) were more common in DSVPTC nodules than in cPTC nodules, corresponding to the infiltrating boundaries and numerous psammoma bodies under the microscope respectively. Most of the DSVPTC cases had a heterogeneous background (80%) and suspicious metastatic cervical lymph nodes (80%) on sonograms. All DSVPTC cases had histopathological metastatic cervical lymph nodes.
The sonographic "snowstorm" pattern indicated DSVPTC with whole-lobe occupation. Hypoechoic solid nodules with vague borders and abundant microcalcifications on sonogram suggested DSVPTC lesion with an ongoing invasion. Regardless of which of the two sonograms was shown, the corresponding DSVPTC lesions were aggressive and required the same attention from the surgeons.
弥漫硬化型甲状腺乳头状癌（DSVPTC）是一种罕见但侵袭性较高的甲状腺乳头状癌亚型，需要采取积极的临床策略。很少有研究关注 DSVPTC 的超声特征，超声在诊断和治疗这种变体中的作用仍不清楚。本研究旨在通过与病理学相关，更准确地识别和了解超声下的 DSVPTC。
回顾分析了 2014 年至 2020 年期间在我院接受手术治疗的 10 例 10 个 DSVPTC 患者的超声特征和组织病理学切片，并与 168 例经典型甲状腺乳头状癌（cPTC）患者的 184 个病变进行比较。
6 例 DSVPTC 病例（60%）在超声上显示“暴风雪”样表现，4 例（40%）仅表现为低回声实性结节。DSVPTC 结节的边界模糊（100.0%比 18.5%，P=0.019）和微钙化丰富（66.7%比 10.9%，P=0.037）比 cPTC 结节更常见，分别对应于显微镜下浸润性边界和大量砂粒体。DSVPTC 结节的超声多表现为不均匀背景（80%）和可疑转移性颈部淋巴结（80%）。所有 DSVPTC 病例均有组织学转移性颈部淋巴结。
超声“暴风雪”样表现提示 DSVPTC 全叶受累。超声表现为低回声实性结节，边界模糊，微钙化丰富，提示 DSVPTC 病变有持续侵袭。无论显示哪种超声表现，相应的 DSVPTC 病变均具有侵袭性，需要外科医生给予同样的关注。
