以突眼起病的儿童非霍奇金淋巴瘤11例

A study on children of non-Hodgkin lymphoma with onset as exophthalmos in eleven cases

目的了解以突眼起病儿童非霍奇金淋巴瘤的临床、病理、影像学特点及其疗效和预后。方法对中山大学附属肿瘤防治中心儿童肿瘤科2004年4月至2010年10月收治的11例以突眼起病的非霍奇金淋巴瘤患儿资料进行临床分析。结果 11例患儿首发症状为单眼眼球突出或双眼眼球突出,有或无其他伴随症状。11例患儿病变部位病理活检均符合淋巴瘤改变。经化疗、手术及放疗等综合治疗,81.8%(9/11)的患儿完全缓解并生存至今。中位随访82个月,死亡2例,均死于肿瘤进展,1例左眼失明,2例右眼失明。1例原发颅内间变大细胞性淋巴瘤的患儿出现颅脑迟发性反射性损伤。结论眼球突出是儿童非霍奇金淋巴瘤的特殊临床表现,疾病早期容易误诊,怀疑本病应尽早行病理活检以明确诊断,如及早确诊并综合治疗,预后良好。

Objective To study clinical pathological and imaging characteristics as well as prognosis of chil-dren of non-Hodgkin lymphoma （NHL） with onset as exophthalmos. Methods The clinical data of eleven children of NHL with onset as exophthalmos who admitted to Cancer Center of Sun Yat-sen University from April 2004 to October 2010 were reviewed. Results The onset of the disease started with exophthalmos in the 11 children were either unilater-al or bilateral protrusion of the eyes, with or without associated symptoms. Diagnoses with NHL were made via patholog-ical biopsy. A median of 82 months follow-up revealed that 9 cases （81.8%） achieved complete remission and sustained survival, but there were 2 cases of dead for the progress of tumor, 1 case of blind left eye and 2 cases of blind right eye. There was delayed brain reflex injury existed in one case of children with primary intracranial large cell lymphoma. Conclusion Exophthalmos is a special clinical manifestation for children NHL, which is prone to be misdiagnosed in the early stages. Therefore, pathological biopsy should be performed as soon as possible when suspected. These diseases are associated with good prognosis after integrating treatment.