Department of Orthopaedics and Rehabilitation, Pennsylvania State University College of Medicine, Penn State Hershey Medical Center, Hershey, PA, USA.
Clin Orthop Relat Res. 2010 Aug;468(8):2288-94. doi: 10.1007/s11999-010-1297-5. Epub 2010 Mar 19.
Chondrosarcomas of the spine constitute 4% to 10% of all primary spinal bone tumors and approximately 70% of the cases occur during the second or third decade of life. Mesenchymal chondrosarcoma is a rare aggressive variant of chondrosarcoma. The prognosis of mesenchymal chondrosarcoma is usually poor with a tendency for late local recurrence and metastasis.
We describe a case of primary mesenchymal chondrosarcoma affecting the L5 vertebra of a 9-year-old girl. The patient underwent a staged circumferential resection of the tumor after three rounds of neoadjuvant chemotherapy. The patient had additional chemotherapy and radiation therapy as an intralesional margin was achieved during the procedure. At 9 years followup, the patient was asymptomatic, neurologically intact, and remained in remission.
We identified only four previously published cases of spinal mesenchymal chondrosarcoma in childhood, two of which had relatively early recurrence and poor survival, and two survived but with only short followup.
As the clinical and radiographic findings of mesenchymal chondrosarcoma are nonspecific, the diagnosis of this rare tumor requires careful histopathologic review of the specimens. We suggest the differential diagnosis of every primary intraspinal tumor include tumors of mesenchymal origin. The prognosis is apparently not uniformly poor.
脊柱软骨肉瘤占所有原发性脊柱骨肿瘤的 4%至 10%,约 70%的病例发生在第二或第三个十年。间充质软骨肉瘤是一种罕见的侵袭性软骨肉瘤变体。间充质软骨肉瘤的预后通常较差,有晚期局部复发和转移的倾向。
我们描述了一例发生在 9 岁女孩 L5 椎体的原发性间充质软骨肉瘤。该患者在三轮新辅助化疗后接受了肿瘤的分期环形切除术。由于在手术过程中实现了瘤内边缘,患者接受了额外的化疗和放疗。在 9 年的随访中,患者无症状,神经完整,处于缓解状态。
我们仅发现了之前发表的四例儿童脊柱间充质软骨肉瘤病例,其中两例复发较早且生存情况较差,两例存活但随访时间较短。
由于间充质软骨肉瘤的临床和影像学表现不具有特异性,因此需要对标本进行仔细的组织病理学检查以确诊这种罕见肿瘤。我们建议将每种原发性脊柱内肿瘤的鉴别诊断包括间充质来源的肿瘤。显然,预后并不完全差。