Abstract:
METHODS: A 14-year-old girl presented with 1 cm large whitened lesion on the ventral surface of the tongue, appeared from 1 month. Past history showed congenital dyserythropoietic anemia type II. The lesion was excised and microscopic and immunohistochemical analyses were compatible with benign Abrikossoff tumor. Total body MRI was negative. After six months the patient presented a second tongue lesion and four months later another large painful lesion in the soft palate, with the same istological diagnosis. In addition, she had other multiple lesions: two apperead at pharyngeal level (not biopsied) that remain stable over time, and one at the pituitary gland.
CONCLUSIONS: Granular cell tumors, with or without multiple lesions, are rare in children. About 50% of cases involve the head and neck region, with the tongue being the most affected site. Therapy is based on the surgical excision of the lesions; however some tumor forms, although their histological aspect of benignity, often have an important infiltrative power, making the therapeutic approach difficult, as in our case.
摘要:
方法:一个14岁的女孩,舌头腹面有1厘米大的白化病变,从1个月出现。既往史显示II型先天性红细胞生成异常性贫血。切除病变,显微镜和免疫组织化学分析与良性Abrikosoff肿瘤相容。全身MRI阴性。六个月后,患者出现了第二个舌头病变,四个月后,软腭又出现了一个大的疼痛病变,具有相同的组织学诊断。此外,她有其他多发性病变:两个在咽部水平(未活检),随着时间的推移保持稳定,一个在脑垂体.
结论:颗粒细胞肿瘤,有或没有多发性病变,在儿童中很少见。大约50%的病例涉及头颈部,舌头是受影响最大的部位。治疗是基于手术切除的病变;然而,一些肿瘤的形式,虽然它们的组织学方面的仁慈,往往有重要的渗透力,使治疗方法变得困难,就像我们的情况一样。