Proton beam therapy for malignancy in Bloom syndrome

Proton beam therapy for malignancy in Bloom syndrome
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质子束治疗布卢姆综合征恶性肿瘤

DOI:
10.1007/s00066-012-0274-1
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发表时间:
2013
期刊:
影响因子:
3.1
通讯作者:
Sakurai H
Sakurai H
中科院分区:
医学2区
文献类型:
--
作者:
Mizumoto M;Hashii H;Senarita M;Sakai S;Wada T;Okumura T;Tsuboi K;Sakurai H

文献摘要

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背景和目的Bloom综合征是一种对放射治疗高度敏感的DNA修复障碍。我们描述了第一例在Bloom综合征患者中使用质子束疗法(PBT)来治疗口咽癌的病例。患者是一位32岁的Bloom综合征女性患者,她被诊断为口咽癌,分期为T2N2bM0低分化鳞状细胞癌。原发灶位于右侧舌根,向右侧咽壁延伸。几个右上区域淋巴结转移阳性。结果我们选择PBT是为了减少对正常组织的剂量。临床靶区体积被定义为原发肿瘤和淋巴结转移的面积加上8 mm的边缘。在用36 Gye(灰当量)分20次(每周4-5次)治疗后,粘膜炎减少了饮食摄入量,并开始静脉高营养。需要终止治疗2.5周以缓解粘膜炎。在33个组分中使用59.4%Gye显著缩小了原发肿瘤的大小,但也引起了需要终止PBT的中度粘膜炎。一个月后,肺转移和乳腺癌发生,患者在PBT后9个月死亡。此时原发肿瘤的缩小得以维持,没有严重的晚期毒性。结论对一例DNA修复缺陷的放射敏感性患者,我们获得了几乎完全的反应,表明质子束治疗的最佳剂量浓度。
Background and purposeBloom syndrome is a DNA repair disorder that is hypersensitive to radiotherapy. We describe the first case in which proton beam therapy (PBT) was used in a patient with Bloom syndrome to treat oropharyngeal cancer.Patients and methodsThe patient was a 32-year-old woman with Bloom syndrome who was diagnosed with oropharyngeal cancer staged as T2N2bM0 poorly differentiated squamous cell carcinoma. The primary tumor was located on the right tongue base and extended to the right lateral pharyngeal wall. Several right upper region lymph nodes were positive for metastases.ResultsWe selected PBT in anticipation of dose reduction to normal tissue. The clinical target volume was defined as the area of the primary tumor and lymph node metastases plus an 8-mm margin. After treatment with 36 GyE (Gray equivalent) in 20 fractions (4–5 fractions per week), dietary intake was decreased by mucositis and intravenous hyperalimentation was started. Termination of treatment for 2.5 weeks was required to relieve mucositis. Administration of 59.4 GyE in 33 fractions markedly reduced the size of the primary tumor, but also caused moderate mucositis that required termination of PBT. One month later, lung metastases and breast cancer developed and the patient died 9 months after PBT. At this time the reduction in size of the primary tumor was maintained without severe late toxicity.ConclusionWe obtained almost complete response for a radiosensitive patient with a deficiency of DNA repair, indicating the excellent dose concentration of proton beam therapy.