Shiohama Tadashi, Ochiai Hidemasa, Hishiki Tomoro, Yoshida Hideo, Kohno Yoichi
Department of Pediatrics, Chiba University Graduate School of Medicine, Chiba, Japan.
Pediatr Int. 2014 Aug;56(4):608-10. doi: 10.1111/ped.12292.
Langerhans cell histiocytosis (LCH) is a rare proliferative disease accompanied by the accumulation of pathological Langerhans cells, which often spreads into multi-site and multi-organ systems. We here describe a girl with a history of Kawasaki disease and cervical lymphadenopathy who presented with occipital LCH. Adrenal tumor was detected on staging evaluation of LCH and was diagnosed as neuroblastoma on resection using laparoscopic surgery. Neither tumor relapsed following chemotherapy for LCH and resection of neuroblastoma. Although LCH often spreads into multi-organ lesions, invasive biopsy may be needed for tumors with atypical localization for LCH in consideration of the synchronous occurrence of malignancies.
朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的增殖性疾病,伴有病理性朗格汉斯细胞的积聚,常累及多部位和多器官系统。我们在此描述一名患有川崎病和颈部淋巴结病病史的女孩,她出现了枕部LCH。在LCH的分期评估中发现了肾上腺肿瘤,经腹腔镜手术切除后诊断为神经母细胞瘤。LCH化疗和神经母细胞瘤切除术后,两种肿瘤均未复发。尽管LCH常累及多器官病变,但考虑到恶性肿瘤的同步发生,对于LCH非典型定位的肿瘤可能需要进行侵入性活检。