Di Giannatale Angela, Colletti Marta, Russo Ida, Ferruzzi Valentina, Dell' Anna Vito Andrea, Cozza Raffaele, Colafati Giovanna Stefania, Messina Raffaella, Mastronuzzi Angela, De Vito Rita, Milano Giuseppe Maria
Department of hematology/Oncology and Stem Cell Transplantation, Bambino Gesù Children's Hospital, IRCCS, Rome - Italy.
Department of Pediatrics, University of Perugia, Perugia - Italy.
Tumori. 2017 Nov 15;103(Suppl. 1):e66-e72. doi: 10.5301/tj.5000689.
Mesenchymal chondrosarcoma (MCS) is an aggressive variant of chondrosarcoma and is a rare tumor, particularly within the pediatric population. Commonly, MCS originates in the bone, but it can also arise in extraskeletal sites, such as the brain and the intraspinal area. Due to the rarity of this tumor, there are no guidelines for its optimal treatment.
We report a case of intradural extramedullary MCS, located at the T11-T12 level, in a 14-year-old male. The tumor was documented by magnetic resonance imaging and treated with gross total resection (GTR) without adjuvant treatment. We further reviewed the relevant pediatric literature and discussed the management and outcome of intracranial and intraspinal MCS.
The patient's follow-up showed no evidence of disease 2 years from diagnosis. A total of 51 cases of intracranial and intraspinal MCS have been reported (24 intraspinal and 27 intracranial). Recurrence has been described in only 4 patients with intraspinal MSC, and among them 3 received adjuvant chemotherapy and radiotherapy. GTR seems to reduce the risk of recurrence and, due to a higher cancer-mortality rate for these patients, adjuvant chemotherapy and radiotherapy are recommended in case aggressive surgery is not possible.
According to our single experience, we would suggest that adjuvant therapy might be unnecessary in cases where a localized MCS undergoes GTR. Chemotherapy and radiotherapy should be recommended when GTR cannot be obtained. Further studies are needed to investigate a standard treatment approach for this rare tumor.
间叶性软骨肉瘤（MCS）是软骨肉瘤的一种侵袭性变异型，是一种罕见肿瘤，在儿童群体中尤为少见。通常，MCS起源于骨骼，但也可发生于骨骼外部位，如脑和脊髓内区域。由于该肿瘤罕见，尚无关于其最佳治疗的指南。
我们报告一例位于T11 - T12水平的硬脊膜内髓外MCS病例，患者为一名14岁男性。通过磁共振成像记录该肿瘤，并采用全切除（GTR）治疗，未进行辅助治疗。我们进一步回顾了相关的儿科文献，并讨论了颅内和脊髓内MCS的管理及预后。
患者随访显示，自诊断起2年无疾病证据。共报告了51例颅内和脊髓内MCS病例（24例脊髓内和27例颅内）。仅4例脊髓内MCS患者出现复发，其中3例接受了辅助化疗和放疗。GTR似乎可降低复发风险，并且由于这些患者的癌症死亡率较高，若无法进行积极手术，则建议进行辅助化疗和放疗。
根据我们的单一经验，我们建议对于局限性MCS行GTR的病例，辅助治疗可能不必要。当无法实现GTR时，应推荐化疗和放疗。需要进一步研究以探讨这种罕见肿瘤的标准治疗方法。
