成人腺泡状横纹肌肉瘤11例临床病理学分析

摘要

Aim To study the clinicopathological features ,immunophenotype and differential diagnosis of alveolar thabdomyosarcoma in adults( ARMS) . Methods The clinical, pathologic and immunohistochemical features were evaluated in 11 cases of ARMS in adults.Results 11 cases of ARMS in adults were retrieved from the pathological archive of this hospital. 7cases were female.4 cases male , age ranging from 19 t0 60 years( median ,41 year) . Histologically , all the tumors were mainly composed of classic form. In the classic form ,it composed of distinctive nests of primitive-appearing round cells , which grew in a discohesive fashion .surrounded by hyalinized and pseudoalvelar pattern. Solid forms of ARMS lacked the prominent nested pattern and cellular discohesion seen in classic form. Immunohistochemically ,the tumor cells were positive for Vimentin myogenin. CD56, Syn , CD99 , S-1OO , desmin , MyoDI were positive in most of the cases. The remaining antibodies ( CK . CD45 ) were all negative. Follow-up information was available in all the cases , ranging from 6 months t0 3 years , revealed that 4 patients died of the disease and 3 patients developed local recurrences. Conclusion ARMS is a rare neoplasm in adults ,and almost commonly occur m male and female , with preferential involvement in the head and neck region. It appears a more aggressive clinical course in adults. Morphologically , ARMS in adults should he differentiated from other small round tumors.%目的 探讨成人腺泡状横纹肌肉瘤(alveolar rhabdomyosarcoma,ARMS)的临床病理学特征、免疫表型和鉴别诊断.方法 回顾性分析11例成人ARMS的临床资料、病理学形态和免疫组化标记结果.结果 11例患者中7例女性,4例男性.年龄 19～60岁,平均41岁.组织学上可见特征性的腺泡状结构,9例经典型均可见部分的实体性区域.1例实体型者由一致的实性细胞巢组成,不见腺泡状结构.1例胚胎性-腺泡状混合型在经典的ARMS的局部区域可见胚胎性横纹肌肉瘤的形态.免疫组化标记显示瘤细胞均表达myogenin、CD56、Vimentin 多数表达Syn(66.7%)、S100(55.6%)、CD99(50%)、desmin(87.5%)、MyoD1(33.3%),均不表达CK、 CD45.术后随访6个月～3年,4例死亡,3例复发.结论 成人ARMS少见,男女均好发,头颈部是最好发部位,具有较强的侵袭性行为.形态学上应于多种小圆细胞肿瘤及透明细胞肿瘤相鉴别.