未经证实:视网膜母细胞瘤(RB)是婴儿期和儿童期最常见的眼内恶性肿瘤。这项研究评估了临床和影像学特征,治疗,复发RB伴四肢骨转移且无中枢神经系统受累的患者的预后。
UNASSIGNED:回顾性分析2015年1月至2021年1月北京同仁医院儿科确诊的5例RB伴四肢骨转移患者的临床资料。
UNASISIGNED:包括3名男性和2名女性(7只眼睛:E组4只,D组各1只,C,andB).最常见的症状是疼痛。3例患者有骨髓和淋巴结转移。3例患者为单发,2例患有多发性骨骼病变。涉及的主要骨骼是股骨,肱骨,距骨,还有尺骨.还观察到骨髓和皮质的同时受累。4例血清神经元特异性烯醇化酶(NSE)水平显着升高,1例略有升高;初次静脉化疗导致NSE水平降低,骨病变逐渐消退。2例患者在随访时死亡,3例完全缓解。统计分析结果显示前房侵犯与预后相关,静脉化疗后血清NSE水平下降有显著性差异。
UNASSIGNED:有必要对RB患者进行定期的终身随访,以及早发现骨转移。前房侵犯可能是一个危险因素。骨髓和皮质的同时受累是RB骨转移图像中的特征性表现。多学科治疗尤其是静脉化疗是有用的,至少在开始的时候。
UNASSIGNED: Retinoblastoma (RB) is the most common intraocular malignancy in infancy and childhood. This study evaluated the clinical and imaging features, treatment, and prognosis of patients with recurrent RB with limb bone metastases and without central nervous system involvement.
UNASSIGNED: The clinical data of five patients with RB with limb bone metastases who were diagnosed at the Department of Pediatrics in Beijing Tongren Hospital between January 2015 and January 2021 were analyzed retrospectively.
UNASSIGNED: Three males and two females were included (seven eyes: four group E and one each of group D, C, and B). The most common symptom was pain. Three patients had bone marrow and lymph node metastases. Three patients had single and two had multiple skeletal lesions. The main bones that were involved were the femur, humerus, talus, and ulna. The simultaneous involvement of the bone marrow and cortex was also observed. Serum neuron-specific enolase (NSE) levels were significantly elevated in four cases and slightly elevated in one case; primary intravenous chemotherapy resulted in a decrease in NSE levels and the gradual resolution of the bone lesions. Two patients died at the time of follow-up and three were in complete remission. The results of the statistical analysis showed that anterior chamber invasion was correlated with prognosis, and there was significant difference in the decrease in the serum NSE levels after intravenous chemotherapy.
UNASSIGNED: Regular lifelong follow-up of patients with RB is warranted to identify bone metastases earlier. Anterior chamber invasion may be a risk factor. The simultaneous involvement of the bone marrow and cortex is characteristic manifestations in images of RB with bone metastases. Multidisciplinary treatment especially intravenous chemotherapy is useful, at least at the beginning.