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具有纤维瘤病/筋膜炎样/韧带样型基质的甲状腺乳头状癌:伴有细胞学和分子研究的罕见亚型的病例报告。

Papillary Thyroid Carcinoma with Fibromatosis/Fasciitis-Like/Desmoid-Type Stroma: Case Report of a Rare Subtype with Cytological and Molecular Study.

机构信息

Department of Laboratory Medicine and Pathology, Hamad Medical Corporation, Doha, Qatar.

出版信息

Head Neck Pathol. 2024 Oct 22;18(1):111. doi: 10.1007/s12105-024-01720-8.

Abstract

BACKGROUND

Papillary thyroid carcinoma (PTC) with fibromatosis/fasciitis-like/desmoid-type stroma is a rare subtype of PTC,characterized by two distinct components: a classic papillary carcinoma component and a spindle cell proliferationresembling fibromatosis or nodular fasciitis. This stromal component adds a unique dimension to the tumor'spathology, making diagnosis more challenging and potentially leading to misclassification.

CASE PRESENTATION

We present a case of this rare entity which contributes to the growing body of literature by providing additionalmolecular data, which may shed light on the biological behaviour of the fibromatosis-like stroma and its relationshipwith the papillary carcinoma component. This case underscores the importance of recognizing this subtype, as itsspindle cell proliferation could be mistaken for a separate neoplasm or reactive process, resulting in inappropriatemanagement.

CONCLUSIONS

Increased awareness of this entity will help pathologists avoid diagnostic pitfalls and guide clinicians in developingmore precise treatment plans, addressing both the malignant papillary component and the unique stromal features.This case further enriches the current understanding of the heterogeneity of PTC and highlights the need fortailored management strategies in rare subtypes.

摘要

背景

伴纤维母细胞瘤样/筋膜炎样/硬纤维瘤样间质的甲状腺乳头状癌(PTC)是 PTC 的一种罕见亚型,其特征为两种截然不同的成分:经典的甲状腺乳头状癌成分和类似于纤维母细胞瘤或结节性筋膜炎的梭形细胞增生。这种间质成分为肿瘤的病理学增加了一个独特的维度,使得诊断更具挑战性,并可能导致误诊。

病例介绍

我们提出了一个该罕见实体的病例,通过提供额外的分子数据,为不断增加的文献做出了贡献,这些数据可能揭示了纤维母细胞瘤样间质的生物学行为及其与甲状腺乳头状癌成分的关系。该病例强调了识别这种亚型的重要性,因为其梭形细胞增生可能被误认为是另一种肿瘤或反应性过程,从而导致不适当的处理。

结论

提高对这种实体的认识将帮助病理学家避免诊断陷阱,并指导临床医生制定更精确的治疗计划,既要考虑恶性的甲状腺乳头状癌成分,也要考虑独特的间质特征。该病例进一步丰富了对 PTC 异质性的现有认识,并强调了在罕见亚型中需要制定针对性的管理策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8fa7/11496448/812a51e9ce18/12105_2024_1720_Fig1_HTML.jpg

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