朗格汉斯细胞肿瘤4例临床报道并文献复习

Langerhans cell neoplasms:4 cases report with literature review

目的:探讨朗格汉斯细胞肿瘤临床表现、病理特点、治疗和预后。方法:描述一组成人朗格汉斯细胞肿瘤[包括3例朗格汉斯细胞增多症(LCH)和1例朗格汉斯细胞肉瘤(LCS)]患者的临床、病理表现、治疗情况等,并复习相关文献。结果:3例LCH患者分别为29岁女性、20岁男性以及35岁男性,临床受累脏器包括骨(2/3)、肝(2/3)、下丘脑-垂体(尿崩症)(2/3)、皮肤(1/3),淋巴结(1/3),甲状腺(1/3)、肺(1/3)。免疫组化为CD1 a(+)、S-100(+),2例患者经4个疗程化疗,1例达到部分缓解,另1例无效。1例LCS患者为77岁女性,以颈部淋巴结肿大起病,淋巴结病理诊断为LCS,病情进展迅速,多脏器受累,死于多脏器功能衰竭。结论:成人LCH少见,临床表现不特异,容易误诊、漏诊,治疗需个体化。LCS临床罕见,侵袭性强,进展快,预后差。

Objective： To describe the clinical and pathological features, treatment and prognosis of langerhans cell neoplasms. Methods ： A group of patients was described, including 3 cases of langerhans cell histiocytosis （ LCH）, and 1 case of langerhans cell sarcoma （LCS）. The related literatures were reviewed. Results： Three patients with LCH were 29 - year - old woman, 20 - year - old man and 35 - year - old man. The involved organs including skeleton （ 2/3 ） , liver （2/3）, hypothalamus - pituitary gland （ diabetes insipidus） （2/3） , skin （ 1/3 ） , lymph node （ 1/3 ） , thyroid gland（ 1/3 ） ,lung（ 1/3 ）. Pathological diagnosis of biopsy was positive for CDla and S - 100. 2 patients received 4 cycles of chemotherapy. One achieved partial remission, the other had no response. The patient with LCS was a 77 - year - old woman, who exhibited lymphadenopathy and was diagnosed by lymph node biopsy. She was involved in several organs, aggravated rapidly and died of multiple organs failure. Conclusion： LCH is not common in adult, with no special clinical manifestations and is easy of diagnostic errors and missed diagnosis. The treatment should be individualization. LCS is rare, with intensive invasion and aggravated rapidly. The prognosis is poor.