Chen H H, Fong L, Su I J, Ting L L, Hong R L, Leung H W, Lui L T
Department of Radiation Oncology, National Cheng Kung University Hospital, Tainan, Taiwan.
Radiother Oncol. 1996 Jan;38(1):1-6. doi: 10.1016/0167-8140(95)01668-6.
Lethal midline granuloma (LMG) is characterized by progressive ulceration and destruction of the midfacial tissue. It occurs more frequently in Oriental than in Western populations. Because of the progress in clinical pathology and immunohistochemistry, most cases have been proven to be malignant lymphomas, especially of T-cell lineage. We describe 92 cases of lethal midline granuloma or centrofacial malignant lymphoma in the period 1959-1993. All received complete courses of radiotherapy. Twenty of them also received combination chemotherapy. Thirty-six cases had specimens available for immunohistochemical study; 25 (69%) of these had a T-cell phenotype, and 6 (17%) were of B-cell lineage. The dose to the nasal region was in the range of 3000-7500 cGy in 11-58 days, and to the neck 3000-6400 cGy in 11-48 days. The overall survival rate for the LMGs was 59.5% at 5 years and 56.2% at 10 years (Kaplan-Meier). Combined chemotherapy seemed not to improve the overall survival in this study (p = 0.63), but the patient number was too small to make a firm conclusion. Based on the results of this study, we recommend a dose of 4500-5000 cGy to the midfacial region, since a higher dosage did not improve the treatment results (p = 0.88). Irradiation has a definite role in good locoregional control of this disease. The recent clarification of the disease nature and the recognition of the background clinicopathological features should provide valuable information for future patient management and prospective studies.
致死性中线肉芽肿（LMG）的特征是面部中部组织进行性溃疡和破坏。它在东方人群中的发病率高于西方人群。由于临床病理学和免疫组织化学的进展，大多数病例已被证实为恶性淋巴瘤，尤其是T细胞谱系。我们描述了1959年至1993年期间的92例致死性中线肉芽肿或面部中心恶性淋巴瘤病例。所有患者均接受了完整疗程的放疗。其中20例还接受了联合化疗。36例有标本可用于免疫组织化学研究；其中25例（69%）具有T细胞表型，6例（17%）为B细胞谱系。鼻腔区域的照射剂量在11至58天内为3000 - 7500 cGy，颈部在11至48天内为3000 - 6400 cGy。LMG患者的5年总生存率为59.5%，10年总生存率为56.2%（Kaplan - Meier法）。在本研究中，联合化疗似乎并未提高总生存率（p = 0.63），但病例数过少，无法得出确切结论。基于本研究结果，我们建议对面部中部区域给予4500 - 5000 cGy的剂量，因为更高剂量并未改善治疗效果（p = 0.88）。放疗在该疾病的良好局部控制中具有明确作用。最近对该疾病性质的阐明以及对背景临床病理特征的认识应为未来的患者管理和前瞻性研究提供有价值的信息。
