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儿童和青少年骨软骨肉瘤

Chondrosarcoma of bone in children and adolescents.

作者信息

Kaneuchi Yoichi, Fujiwara Tomohiro, Tsuda Yusuke, Yoshida Shinichirou, Stevenson Jonathan D, Abudu Adesegun

机构信息

Royal Orthopaedic Hospital NHS Foundation Trust, Birmingham, UK.

出版信息

J Child Orthop. 2020 Aug 1;14(4):330-334. doi: 10.1302/1863-2548.14.200046.

DOI:10.1302/1863-2548.14.200046
PMID:32874367
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7453173/
Abstract

PURPOSE

Chondrosarcomas typically present in adults during the fifth to seventh decades and are rare in young patients. The biological behaviour and oncological outcomes may be different in children and adolescents.

METHODS

We retrospectively evaluated the outcomes of all patients with chondrosarcoma of bone who were younger than 18 years of age at the time of diagnosis and were treated at our centre between 1995 and 2018.

RESULTS

The 15 consecutive patients studied included nine male and six female cases, with a mean age at diagnosis of 13 years (7 to 17). The median follow-up was 117 months (30 to 277). The tumours were primary and secondary in ten and five patients, respectively. The tumours were central in 13 and surface in two patients. The tumour locations were the humerus in five, digits in five, femur in three, radius in one and pelvis in one patient. The histological grades were grade I in seven, grade II in seven and grade III in one patient. The surgical treatments were limb salvage in ten patients and ray amputation in five patients. The surgical margins were wide in eight, marginal in two and intralesional in five patients. All the patients were alive and continuously free of disease at the time of the last follow-up. No patient developed metastases or local recurrence.

CONCLUSION

Chondrosarcoma of bone in children and adolescent patients has a very good prognosis and is less aggressive compared with published outcomes in older patients.

LEVEL OF EVIDENCE

IV.

摘要

目的

软骨肉瘤通常在成人的第五至第七个十年出现,在年轻患者中罕见。儿童和青少年的生物学行为和肿瘤学结局可能有所不同。

方法

我们回顾性评估了1995年至2018年期间在我们中心接受治疗、诊断时年龄小于18岁的所有骨软骨肉瘤患者的结局。

结果

连续研究的15例患者中,男性9例,女性6例,诊断时的平均年龄为13岁(7至17岁)。中位随访时间为117个月(30至277个月)。肿瘤分别为原发性和继发性,各有10例和5例。13例肿瘤位于中央,2例位于表面。肿瘤部位分别为:肱骨5例,手指5例,股骨3例,桡骨1例,骨盆1例。组织学分级为I级7例,II级7例,III级1例。手术治疗包括10例保肢手术和5例截指手术。手术切缘8例为广泛切缘,2例为边缘切缘,5例为囊内切缘。在最后一次随访时,所有患者均存活且无疾病复发。无患者发生转移或局部复发。

结论

儿童和青少年骨软骨肉瘤的预后非常好,与老年患者已发表的结局相比,侵袭性较小。

证据级别

IV级。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/61c2/7453173/aecc9396b9ca/jco-14-330-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/61c2/7453173/aecc9396b9ca/jco-14-330-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/61c2/7453173/aecc9396b9ca/jco-14-330-g0001.jpg

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Chondrosarcoma in a paediatric population: a study of 247 cases.儿童人群中的软骨肉瘤:一项247例病例的研究。
J Child Orthop. 2019 Feb 1;13(1):89-99. doi: 10.1302/1863-2548.13.180109.
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The role of surgical margins in chondrosarcoma.软骨肉瘤中外科边界的作用。
Eur J Surg Oncol. 2018 Sep;44(9):1412-1418. doi: 10.1016/j.ejso.2018.05.033. Epub 2018 Jun 8.
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The role of grade in local recurrence and the disease-specific survival in chondrosarcomas.在软骨肉瘤中,分级在局部复发和疾病特异性生存中的作用。
Bone Joint J. 2018 May 1;100-B(5):662-666. doi: 10.1302/0301-620X.100B5.BJJ-2017-1243.R1.
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A diagnostic approach to bone tumours.骨肿瘤的诊断方法。
Pathology. 2017 Dec;49(7):675-687. doi: 10.1016/j.pathol.2017.08.003. Epub 2017 Oct 27.
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Mesenchymal chondrosarcoma: A Japanese Musculoskeletal Oncology Group (JMOG) study on 57 patients.间叶性软骨肉瘤:日本肌肉骨骼肿瘤学组(JMOG)对57例患者的研究。
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UK guidelines for the management of bone sarcomas.英国骨肉瘤管理指南。
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