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婴儿纤维肉瘤辅助治疗的进展

Advances in the adjuvant treatment of infantile fibrosarcoma.

作者信息

Ferguson William S

机构信息

Division of Pediatric Hematology-Oncology, Rhode Island Hospital, Providence 02903, USA.

出版信息

Expert Rev Anticancer Ther. 2003 Apr;3(2):185-91. doi: 10.1586/14737140.3.2.185.

Abstract

Infantile fibrosarcoma is a rare soft tissue tumor, predominately affecting young infants. It grows rapidly and is locally infiltrative but rarely metastases. Complete surgical removal is usually curative but is impossible in some patients and would result in significant functional or cosmetic consequences in many others. Neoadjuvant chemotherapy will cause many tumors to shrink significantly, allowing less mutilating surgical resections to be performed--this is the current recommendation where immediate surgical removal cannot be accomplished without unacceptable morbidity. In contrast, there is no defined role for adjuvant chemotherapy or radiation following complete surgical resection. Although there is a significant risk of local recurrence, most of these can be successfully treated with further surgery and the overall survival rate exceeds 90%.

摘要

婴儿纤维肉瘤是一种罕见的软组织肿瘤,主要影响幼儿。它生长迅速,具有局部浸润性,但很少发生转移。完整的手术切除通常可治愈,但在一些患者中无法实现,且在许多其他患者中会导致严重的功能或美容后果。新辅助化疗会使许多肿瘤显著缩小,从而能够进行创伤较小的手术切除——这是目前的推荐方法,即在不造成不可接受的发病率的情况下无法立即进行手术切除时采用。相比之下,在完整手术切除后,辅助化疗或放疗没有明确的作用。虽然局部复发风险很大,但大多数复发可以通过进一步手术成功治疗,总体生存率超过90%。

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