Schröder S, Bay V, Dumke K, Kremens B, Müller-Gärtner H W, Böcker W, Kastendieck H
Institute of Pathology, University of Hamburg, Federal Republic of Germany.
Virchows Arch A Pathol Anat Histopathol. 1990;416(4):367-71. doi: 10.1007/BF01605298.
The recently published second edition of the WHO classification of thyroid tumours describes the diffuse sclerosing papillary carcinoma (DSPC) as a specific variant of papillary thyroid cancer (PC). Besides several histological hallmarks, this rare tumour is characterized by its occurrence in young individuals and is thought to have a less favourable prognosis than PC in general. The observations on two examples of this tumour presented herein, however, are at variance at this assumption. The neoplasms occurred in a 10 year old girl and a 34 year old woman. Each time, diffuse involvement of both thyroid lobes and bilateral cervical lymphadenopathy were seen. In one case, the carcinoma extended into the cervical soft tissue. Follow-up disclosed both patients to be without evidence of disease 2 and 13 years, respectively, after thyroid surgery. Immunocytochemically, both thyroid primaries as well as 7 other cases of DSPC reported in the literature showed dense accumulations of S-100 protein positive dendritic/Langerhans cells. Such infiltrations have been demonstrated to be correlated with a benign clinical course of PC. It is thus suggested that DSPC behaves similarly or even less aggressively than PC in general, at least if prominent Langerhans cell infiltration is present.
世界卫生组织甲状腺肿瘤分类的最新第二版将弥漫性硬化性乳头状癌（DSPC）描述为甲状腺乳头状癌（PC）的一种特殊变体。除了一些组织学特征外，这种罕见肿瘤的特点是发生于年轻人，并且一般认为其预后比普通PC更差。然而，本文所展示的该肿瘤两个病例的观察结果与这一假设不符。肿瘤分别发生在一名10岁女孩和一名34岁女性身上。每次均可见双侧甲状腺叶弥漫性受累及双侧颈部淋巴结病。在其中1例中，癌肿扩展至颈部软组织。随访发现，两名患者在甲状腺手术后分别有2年和13年无疾病证据。免疫细胞化学检测显示，本文报道的这两例甲状腺原发肿瘤以及文献中报道的其他7例DSPC均有密集聚集的S - 100蛋白阳性树突状/朗格汉斯细胞。已证实这种浸润与PC的良性临床病程相关。因此提示，至少在存在显著朗格汉斯细胞浸润的情况下，DSPC的行为与普通PC相似，甚至侵袭性更小。
