Patiroglu Turkan, Isik Bilgen, Unal Ekrem, Canoz Ozlem, Deniz Kemal, Kosemehmetoglu Kemal, Karakukcu Musa, Ozdemir Mehmet Akif
Division of Pediatric Hematology and Oncology, Department of Pediatrics, Faculty of Medicine, Erciyes University, 38039, Talas, Kayseri, Turkey,
Childs Nerv Syst. 2014 Oct;30(10):1737-41. doi: 10.1007/s00381-014-2443-2. Epub 2014 Jun 11.
Widespread alveolar rhabdomyosarcoma (ARMS) with bone marrow involvement and with an unknown primary tumor, especially presenting with acute tumor lysis syndrome can be easily misdiagnosed as a hematological malignancy. Furthermore, brain metastasis of ARMS is rare seen in children.
Herein, we report a 14-year-old boy presenting with acute tumor lysis syndrome due to bone marrow invasion of ARMS, who was diagnosed after abdominal paraaortic lymph node biopsy. Despite radiological and nuclear medicine imaging, the primary tumor site could not be found. He was treated with vincristine, topotecan, and cyclophosphamide for 42 weeks. Six months after the completion of treatment, he suffered from severe headache, blurred vision, right hemiplegia, and severe bone pain. Cranial magnetic resonance imaging showed multiple hemorrhagic infarctions. Brain biopsy showed brain metastasis with PAX3-FKHR fusion transcript.
The clinicians must be vigilant about solely brain metastasis in ARMS without additional metastasis.
广泛播散的伴有骨髓受累且原发肿瘤不明的肺泡型横纹肌肉瘤(ARMS),尤其是出现急性肿瘤溶解综合征时,很容易被误诊为血液系统恶性肿瘤。此外,ARMS的脑转移在儿童中罕见。
在此,我们报告一名14岁男孩,因ARMS骨髓浸润出现急性肿瘤溶解综合征,经腹主动脉旁淋巴结活检后确诊。尽管进行了放射学和核医学成像检查,仍未找到原发肿瘤部位。他接受了长春新碱、拓扑替康和环磷酰胺治疗42周。治疗结束6个月后,他出现严重头痛、视力模糊、右侧偏瘫和严重骨痛。头颅磁共振成像显示多发出血性梗死。脑活检显示脑转移伴PAX3-FKHR融合转录本。
临床医生必须警惕ARMS单纯脑转移而无其他转移的情况。