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转移性软骨母细胞瘤的组织学与生物学特性。1例病例报告并文献复习

Histology and biology of metastatic chondroblastoma. Report of a case with a review of the literature.

作者信息

Kunze E, Graewe T, Peitsch E

出版信息

Pathol Res Pract. 1987 Feb;182(1):113-23. doi: 10.1016/S0344-0338(87)80151-6.

Abstract

This case report of a metastasizing chondroblastoma with a review of the literature was undertaken to gain a better understanding of the biologic behavior of this exceedingly rare tumor and thus to facilitate its clinical management. The lung was by far the most frequent metastatic site. Thus, all 7 patients with a proven metastatic chondroblastoma recorded up to now including the present case had developed multiple pulmonary metastases. The interval between the initial diagnosis of the primary and manifestation of lung metastases proved to be long, with a mean of 8.4 years. The average survival time was at least 12.3 years. The mean interval between diagnosis of metastatic disease and death amounted to at least 6.3 years. The histomorphologic features of metastatic chondroblastoma, its local recurrences and of the metastatic lesions differed in no way from conventional chondroblastomas. Because of the lack of cellular criteria of malignancy it is impossible to predict the potential biologic behavior of chondroblastomas, in particular with respect to their ability to metastasize. However, the presence of tumor emboli in the primary lesion is highly suggestive of a subsequent development of metastatic disease. The delayed induction of hematogenous metastases is best explained by a limited growth potential of the tumor cells. In case of a large primary tumor--especially in flat bones--with soft tissue invasion or in the presence of tumor emboli an aggressive surgical approach is suggested. When lung metastases have developed their surgical removal is recommended to hopefully prolong live expectancy or even to obtain a curative effect.

摘要

本文通过一例转移性软骨母细胞瘤病例报告并结合文献复习,旨在更好地了解这种极为罕见肿瘤的生物学行为,从而促进其临床管理。肺是迄今为止最常见的转移部位。因此,截至目前记录在案的所有7例经证实的转移性软骨母细胞瘤患者(包括本病例)均出现了多发性肺转移。原发性肿瘤初次诊断与肺转移表现之间的间隔时间较长,平均为8.4年。平均生存时间至少为12.3年。转移性疾病诊断与死亡之间的平均间隔时间至少为6.3年。转移性软骨母细胞瘤、其局部复发及转移灶的组织形态学特征与传统软骨母细胞瘤并无差异。由于缺乏恶性细胞标准,无法预测软骨母细胞瘤的潜在生物学行为,尤其是其转移能力。然而,原发性病变中存在肿瘤栓子高度提示随后会发生转移性疾病。肿瘤细胞生长潜力有限最能解释血行转移的延迟发生。对于大型原发性肿瘤——尤其是扁平骨中的肿瘤——伴有软组织侵犯或存在肿瘤栓子时,建议采取积极的手术方法。当出现肺转移时,建议手术切除,有望延长预期寿命甚至获得治愈效果。

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