Schovanec J, Mrácek J, Havlas V, Trc T
Ortopedická klinika 2. LF UK a FN Motol, detská a dospelá ortopedie a traumatologie, Praha.
Acta Chir Orthop Traumatol Cech. 2004;71(4):220-7.
Neoplastic diseases continue to arouse much concern in the general population. This also applies to bone tumors. Until recently, these neoplasms, most frequently occurring in children and adolescents, were considered to have the worst possible prognosis with a minimal opportunity for a successful outcome of treatment. This is a likely reason for the still deeply rooted belief that amputation of the affected limb is necessary and disease prognosis is uncertain, which also applies to Ewing's sarcoma. Only lately could these patients be offered a hope of successful treatment including limb salvage.
In the period from 1984 to mid-2003, 78 patients with Ewing's sarcoma were treated in our department. Their age ranged from 3 to 25 years, with the maximum occurrence between 13 and 17 years. We performed 27 reconstructions, 13 amputations or exarticulations and 24 resections without replacements. In 13 patients, the procedure was restricted to diagnostic biopsy only, because the tumor was inoperable.
The diagnosis was made on the basis of diagnostic biopsy and, subsequently, neoadjuvant chemotherapy was administered. When this resulted in tumor regression, definitive surgery was performed, involving tumor resection and bone replacement either with autograft or allograft, or with an individual prosthesis. When an implant was not necessary for maintaining limb function, a simple resection was carried out. In the patients in whom the tumor failed to regress, amputation or exarticulation was inevitable. Inoperable tumors were managed by megadose chemotherapy and by radiotherapy. The patients after orthopedic surgery receive adjuvant therapy, including bone marrow transplantation in indicated cases.
The evaluation of our 78 patients showed that patient survival is not related to the surgical procedure used. In patients with an early diagnosis and a positive response of the tumor to chemotherapy, the reconstruction procedure appeared to be sufficiently radical in terms of cancer control while preserving limb function; these patients showed neither a significant increase in disease recurrence nor metastatic dissemination. Similar outcomes were also recorded in patients with a simple resection. However, amputation and exarticulation cannot completely be avoided and they are necessary in the patients whose tumor failed to respond to chemotherapy or in whom radical removal of the tumor is not possible. The poorest outcome was recorded in the patients with inoperable tumors, in whom it was not possible to carry out a radical orthopedic operation. The time between the onset of pain and initiation of therapy is also an important factor affecting the treatment outcome. Complications of reconstructive surgery for Ewing's sarcoma recorded in our patient group included osteomyelitis and graft fracture in addition to relapse and metastatic dissemination followed by death.
Reconstructive surgery for Ewing's sarcoma is carried out in our department in patients with a confirmed diagnosis, in whom neoadjuvant therapy has resulted in tumor regression and in whom the extent and site of a tumor permit this sort of procedure. If this is not possible, limb amputation is indicated. If amputation is not possible due to tumor localization, megadose chemotherapy is administered. The results of long-term survival evaluation of our patients undergoing resection and replacement show that the procedure has been sufficiently radical, because no local recurrence or metastatic dissemination followed by death were recorded. Disease recurrence, death, graft osteomyelitis and fracture of a graft inclusive of osteosynthetic material are the complications reported.
This study evaluated the current techniques of treating Ewing's sarcoma, with emphasis on reconstructive surgery leading to limb salvation and maintenance of its full function. The importance of this approach is obvious when we realize that the patients are mostly children and adolescents.
肿瘤性疾病持续引起普通大众的广泛关注。这也适用于骨肿瘤。直到最近，这些最常发生于儿童和青少年的肿瘤仍被认为预后最差，治疗成功的机会微乎其微。这可能是截肢治疗患肢仍被根深蒂固地认为是必要的，且疾病预后不确定这一观念的原因，尤因肉瘤也不例外。直到最近，这些患者才有望获得包括保肢治疗在内的成功治疗。
在1984年至2003年年中期间，我科共治疗了78例尤因肉瘤患者。他们的年龄在3至25岁之间，发病高峰在13至17岁。我们进行了27例重建手术、13例截肢或关节离断手术以及24例未进行置换的切除术。13例患者仅进行了诊断性活检，因为肿瘤无法手术切除。
通过诊断性活检进行诊断，随后给予新辅助化疗。当化疗导致肿瘤缩小后，进行确定性手术，包括肿瘤切除并用自体骨移植、异体骨移植或个体化假体进行骨置换。当维持肢体功能无需植入物时，则进行单纯切除术。对于肿瘤未缩小的患者，截肢或关节离断不可避免。无法手术切除的肿瘤采用大剂量化疗和放疗。骨科手术后的患者接受辅助治疗，包括在适当情况下进行骨髓移植。
对我们的78例患者的评估表明，患者的生存率与所采用的手术方式无关。对于早期诊断且肿瘤对化疗有阳性反应的患者，重建手术在控制癌症方面似乎足够彻底，同时保留了肢体功能；这些患者疾病复发或转移扩散均未显著增加。单纯切除的患者也有类似结果。然而，截肢和关节离断无法完全避免，对于肿瘤对化疗无反应或无法彻底切除肿瘤的患者是必要的。无法手术切除的肿瘤患者预后最差。疼痛出现至开始治疗的时间也是影响治疗结果的一个重要因素。我们患者组中记录的尤因肉瘤重建手术并发症包括骨髓炎和移植骨骨折，此外还有复发、转移扩散及死亡。
我科对确诊的尤因肉瘤患者进行重建手术，这些患者新辅助治疗后肿瘤缩小，且肿瘤范围和部位允许进行此类手术。如果无法进行，则建议截肢。如果因肿瘤位置无法截肢，则给予大剂量化疗。对接受切除和置换的患者进行长期生存评估的结果表明，该手术已足够彻底，因为未记录到局部复发或转移扩散及死亡。报告的并发症包括疾病复发、死亡、移植骨骨髓炎以及包括骨合成材料在内的移植骨骨折。
本研究评估了当前治疗尤因肉瘤的技术，重点是实现保肢并维持其全部功能的重建手术。当我们意识到患者大多是儿童和青少年时，这种方法的重要性就显而易见了。
