多中心骨肉瘤、罗思蒙德-汤姆森综合征与继发性鼻咽非霍奇金淋巴瘤：一例报告并文献复习
Multicentric osteosarcoma, Rothmund-Thomson syndrome, and secondary nasopharyngeal non-Hodgkin's lymphoma: a case report and review of the literature.
作者信息
Spurney C, Gorlick R, Meyers P A, Healey J H, Huvos A G
机构信息
New York Medical College, Valhalla, USA.
出版信息
J Pediatr Hematol Oncol. 1998 Sep-Oct;20(5):494-7. doi: 10.1097/00043426-199809000-00018.
PURPOSE
Rothmund-Thomson syndrome (RTS) is an autosomal recessive disease characterized by poikiloderma, photosensitivity, skeletal deformities, and other changes. It is also associated with an increased risk of malignancies, including osteosarcoma. Sixteen previous cases of RTS and osteosarcoma have been reported.
PATIENT
A patient with RTS in whom multicentric osteosarcoma developed is described. After surgery and chemotherapy, a secondary malignant neoplasm (SMN) developed consistent with nasopharyngeal non-Hodgkin's lymphoma.
RESULTS
The patient was treated with anti-CD20 monoclonal antibody and is in complete remission.
CONCLUSION
This is the first report of a patient with RTS treated for osteosarcoma in whom a SMN developed. Potential factors involved in this SMN include genetic predisposition and previous chemotherapy.
目的
罗思蒙德-汤姆森综合征（RTS）是一种常染色体隐性疾病，其特征为皮肤异色症、光敏性、骨骼畸形及其他变化。它还与包括骨肉瘤在内的恶性肿瘤风险增加有关。此前已报告过16例RTS合并骨肉瘤的病例。
患者
描述了1例发生多中心骨肉瘤的RTS患者。手术和化疗后，出现了与鼻咽非霍奇金淋巴瘤一致的继发性恶性肿瘤（SMN）。
结果
该患者接受了抗CD20单克隆抗体治疗，目前处于完全缓解状态。
结论
这是首例关于接受骨肉瘤治疗的RTS患者发生SMN的报告。该SMN可能涉及的因素包括遗传易感性和既往化疗。
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