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软骨母细胞瘤:多样的组织学表现、潜在的诊断陷阱以及与局部复发相关的临床病理特征。

Chondroblastoma: varied histologic appearance, potential diagnostic pitfalls, and clinicopathologic features associated with local recurrence.

作者信息

de Silva M V Chandu, Reid Robin

机构信息

University Department of Pathology/Scottish Bone Tumour Registry, Western Infirmary, Glasgow, UK.

出版信息

Ann Diagn Pathol. 2003 Aug;7(4):205-13. doi: 10.1016/s1092-9134(03)00048-0.

Abstract

Chondroblastoma is a rare, benign bone tumor. Although it has distinctive clinicopathologic features, its wide morphologic spectrum may pose diagnostic problems. We present the clinicopathologic features of 42 patients (28 males, 14 females; age range, 8 to 66 years), with emphasis on unusual histologic features, potential diagnostic pitfalls, and factors associated with recurrence. Thirty-four tumors were in long bones, with the most common site being the proximal femur. Unusual histologic features included the presence of atypical, epithelioid, spindle, and foamy cells and necrosis and a diffuse basophilic myxoid matrix. Tumors with focal osteoclast-like giant cell rich areas (n = 11), prominent cystic change (n = 8) and extensive fibromyxoid areas (n = 3) resembled giant cell tumors, aneurysmal bone cysts, and chondromyxoid fibromas, respectively. The diagnosis of referring pathologists was inaccurate in 34% of cases. Six patients (14%) had local recurrence. The only clinical feature significantly associated with increased risk of local recurrence was duration of symptoms for less than 6 months (log rank P =.003). None of the histologic features was significantly associated with recurrence. These included worrisome features such as cellular atypia, necrosis, and mitoses. None of the patients had metastases. An increased awareness of the morphologic spectrum of chondroblastomas will enable pathologists to avoid diagnostic pitfalls. We emphasize the need for a combined clinical, radiologic and histologic approach to the diagnosis of chondroblastomas.

摘要

软骨母细胞瘤是一种罕见的良性骨肿瘤。尽管它具有独特的临床病理特征,但其广泛的形态学谱可能会带来诊断问题。我们呈现了42例患者(28例男性,14例女性;年龄范围8至66岁)的临床病理特征,重点关注不寻常的组织学特征、潜在的诊断陷阱以及与复发相关的因素。34个肿瘤位于长骨,最常见的部位是股骨近端。不寻常的组织学特征包括存在非典型、上皮样、梭形和泡沫细胞以及坏死和弥漫性嗜碱性黏液样基质。具有局灶性破骨细胞样巨细胞丰富区域(n = 11)、显著囊性变(n = 8)和广泛纤维黏液样区域(n = 3)的肿瘤分别类似于巨细胞瘤、动脉瘤样骨囊肿和软骨黏液样纤维瘤。转诊病理学家的诊断在34%的病例中不准确。6例患者(14%)出现局部复发。与局部复发风险增加显著相关的唯一临床特征是症状持续时间少于6个月(对数秩检验P = 0.003)。没有任何组织学特征与复发显著相关。这些特征包括令人担忧的特征,如细胞异型性、坏死和有丝分裂。所有患者均无转移。提高对软骨母细胞瘤形态学谱的认识将使病理学家能够避免诊断陷阱。我们强调对软骨母细胞瘤的诊断需要临床、放射学和组织学相结合的方法。

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