Department of Radiation Oncology, Proton Medical Research Center, University of Tsukuba Hospital; Department of Child Health, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan.
Department of Radiation Oncology, Proton Medical Research Center, University of Tsukuba Hospital, Tsukuba, Ibaraki, Japan.
J Cancer Res Ther. 2020 Oct-Dec;16(6):1524-1527. doi: 10.4103/jcrt.JCRT_449_19.
Li-Fraumeni syndrome (LFS) is a genetic disease that is hypersensitive to radiotherapy. Proton therapy (PT) was strongly recommended for pediatric and radiation-sensitive tumors. However, there is little information on PT for LFS. The patient was a 7-year-old girl with LFS who was diagnosed with radiation-induced right shoulder blade osteosarcoma and left chest wall malignant fibrous histiocytoma. Both tumors were in the area that had previously been irradiated (36-45 Gy by photon radiotherapy). Sixty-six GyE in 30 fractions was planned for both tumors. We set the clinical target to the minimum gross tumor volume. To comprehensively assess any adverse events, PT was conducted under hospital administration. Cisplatin was used as simultaneous combination chemotherapy. Although administration of granulocyte-colony stimulating factor was necessary for myelosuppression by chemotherapy, PT was completed without interruption. Acute radiation toxicity was observed as Grade 1 dermatitis. The dermatitis became exacerbated 2 weeks after PT but subsequently improved with conservation treatment alone. Twenty-three months after PT, magnetic resonance imaging showed an increase in the tumor on the right shoulder. A histological examination was not conducted as the family declined, but secondary cancer was suggested rather than recurrent osteosarcoma, as the tumor developed mainly from the soft tissue. Additional surgical treatment and radiotherapy were not indicated, and the patient died of tumor progression and sepsis caused by myelosuppression 27 months after undergoing PT. Up to 23 months after PT, there were no signs of Grade 2 or more late toxicities. This represents the first reported case of PT for a patient with LF to treat radiation-induced secondary cancer.
李-佛美尼综合征（LFS）是一种对放疗敏感的遗传性疾病。质子治疗（PT）强烈推荐用于儿科和对放疗敏感的肿瘤。然而，关于 LFS 的 PT 信息很少。患者为 7 岁女孩，患有 LFS，被诊断为放射性右肩胛骨骨肉瘤和左胸壁恶性纤维组织细胞瘤。两个肿瘤均位于先前接受过放疗（光子放疗 36-45Gy）的区域。计划对两个肿瘤进行 66GyE/30 次分割。我们将临床靶区设定为最小大体肿瘤体积。为了全面评估任何不良事件，PT 在医院管理下进行。顺铂作为同步联合化疗药物。尽管化疗引起的骨髓抑制需要使用粒细胞集落刺激因子，但 PT 仍未中断地完成。急性放射毒性表现为 1 级皮炎。PT 后 2 周，皮炎加重，但随后仅通过保守治疗即可改善。PT 后 23 个月，磁共振成像显示右肩肿瘤增大。由于家庭拒绝，未进行组织学检查，但考虑为继发性癌症而不是复发性骨肉瘤，因为肿瘤主要源自软组织。未进行额外的手术治疗和放疗，PT 后 27 个月患者因骨髓抑制导致肿瘤进展和脓毒症死亡。PT 后 23 个月内，无 2 级或更高级别的迟发性毒性迹象。这是首例报告的 LFS 患者接受 PT 治疗放射性继发性癌症的病例。
