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Chondroblastomas presenting in adulthood: a study of 39 patients with emphasis on histological features and skeletal distribution.

成人期表现的软骨母细胞瘤: 39 例患者的研究,重点是组织学特征和骨骼分布。

  • 影响因子:2.67
  • DOI:10.1111/his.13972
  • 作者列表:"John I","Inwards CY","Wenger DE","Williams DD","Fritchie KJ
  • 发表时间:2020-01-01
Abstract

AIMS:Chondroblastomas (CB) are rare bone tumours that typically arise in the epiphysis/apophysis of long bones in skeletally immature patients. We explore the clinicopathological features of CB presenting in adults. METHODS AND RESULTS:CB in patients ≥20 years of age were retrieved from our institutional archives. Thirty-nine CB were identified (29 male/10 female; aged 20-54 years). Twenty (51%) cases occurred in long tubular bones, 10 (26%) in small bones of the feet, five (13%) in flat bones and four (10%) in the patella. All cases showed classic cytological features of CB, and chondroid matrix was universally present. Calcification was identified in 10 cases (26%), including various combinations of serpiginous (n = 7), punctate (n = 6), classic chicken-wire (n = 4) and psammomatous (n = 2) patterns. Haemosiderin (n = 19), woven bone (n = 13), secondary aneurysmal bone cyst formation (n = 8), foamy macrophages (n = 4), hyalinised vascular spaces (n = 2) and cholesterol clefts (n = 2) were noted. Follow-up information (n = 32, 1-452 months) revealed local recurrence in three patients, all >40 years of age with flat bone origin, one of which developed pulmonary metastases 132 months after initial diagnosis. CONCLUSIONS:CB in patients >20 years of age more frequently involves the short bones of the hands/feet and flat bones compared to those arising in their younger counterparts. A subset may harbour extensive serpiginous or psammomatous calcification rather than the classic chicken-wire pattern. Although the overall local recurrence rate in adulthood is approximately 10%, all three patients with recurrent disease had tumours involving flat bones, suggesting that tumours arising in these sites may behave more aggressively.

摘要

目的: 软骨母细胞瘤 (CB) 是一种罕见的骨肿瘤,通常出现在骨骼不成熟患者的长骨骨骺/突起中。我们探讨成人CB的临床病理特征。 方法和结果: 从我们的机构档案中检索 ≥ 20 岁患者的CB。鉴定出 39 例CB (29 例男性/10 例女性; 年龄 20-54 岁)。20 例 (51%) 发生于长管状骨,10 例 (26%) 发生于足部小骨,5 例 (13%) 发生于扁骨,4 例 (10%) 发生于髌骨。所有病例均表现为典型的CB细胞学特征,软骨样基质普遍存在。钙化 10 例 (26%),包括各种serpious组合 (n = 7) 、点状 (n = 6) 、经典chicken-wire (n = 4) 和砂粒 (n = 2) 模式。血铁质素 (n = 19) 、编织骨 (n = 13) 、继发性动脉瘤样骨囊肿形成 (n = 8) 、泡沫状巨噬细胞 (n = 4) 、观察到透明血管间隙 (n = 2) 和胆固醇裂隙 (n = 2)。随访信息 (n = 32,1-452 个月) 发现 3 例患者局部复发,均> 40 岁,骨起源平坦,其中 1 例在初诊后 132 个月出现肺转移。 结论: 与年轻同行相比,年龄> 20 岁患者的CB更常涉及手/足的短骨和扁骨。一个子集可能存在广泛的serpious或psamomatous钙化,而不是经典的chicken-wire模式。虽然成年期的总体局部复发率约为 10%,但所有 3 例复发性疾病患者的肿瘤均累及扁骨,提示这些部位的肿瘤表现可能更积极。

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影响因子:1.41
发表时间:2020-03-01
DOI:10.1177/1078155219842277
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骨肿瘤方向

骨肿瘤是发生于骨骼或其附属组织的肿瘤。有良性,恶性之分,良性骨肿瘤易根治,预后良好,恶性骨肿瘤发展迅速,预后不佳,死亡率高。

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