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肝母细胞瘤伴贝克威思-维德曼综合征和半身肥大。

Hepatoblastoma associated with Beckwith-Wiedemann syndrome and hemihypertrophy.

作者信息

Hamada Y, Takada K, Fukunaga S, Hioki K

机构信息

Second Department of Surgery, Kansai Medical University, 10-15, Fumizono, Moriguchi City, Osaka 570-8507, Japan.

出版信息

Pediatr Surg Int. 2003 Apr;19(1-2):112-4. doi: 10.1007/s00383-002-0734-2. Epub 2003 Mar 2.

Abstract

Both Beckwith-Wiedemann syndrome (BWS) and hemihypertrophy (HH) have been recognized to be overgrowth syndromes associated with an increased risk of cancer. We report an infant with hepatoblastoma associated with both BWS and HH in whom high serum alpha-fetoprotein (AFP) levels persisted even after complete tumor resection with no tumor recurrence. This phenomenon might be partly due to the nature of the proliferative disease. It is important to recognize that in some infants with BWS prolonged high serum AFP levels mimic the existence of a tumor, and that treatment should be based not only on AFP measurement, but also on repeated radiologic imaging.

摘要

贝克威思-维德曼综合征(BWS)和半侧肥大(HH)均被认为是与癌症风险增加相关的过度生长综合征。我们报告了一名患有肝母细胞瘤的婴儿,该婴儿同时患有BWS和HH,即使在肿瘤完全切除且无肿瘤复发后,其血清甲胎蛋白(AFP)水平仍持续升高。这种现象可能部分归因于增殖性疾病的性质。重要的是要认识到,在一些患有BWS的婴儿中,血清AFP水平长期升高会让人误以为存在肿瘤,而且治疗不仅应基于AFP测量,还应基于反复的影像学检查。

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