小肠原发组织细胞肉瘤临床病理观察

Histocytic sarcoma of small intestine: a clinicopathological analysis

目的探讨小肠组织细胞肉瘤(HS)的临床病理特征及鉴别诊断。方法分析1例原发于小肠的HS临床病理特征、免疫组化结果进行回顾性分析,并复习相关文献。结果患者男性64岁,临床表现为腹痛、持续黑便,CT:表现为肠套叠,似见软组织密度影,不除外合并小肠占位。镜检见小肠黏膜下肿瘤呈弥漫片状排列,细胞圆形、卵圆形,胞质丰富、嗜酸,核泡状、多型性,核仁明显,背景见急慢性炎症细胞浸润。免疫组化:vimentin、CD68、CD163、Lys、CD10 (+);CKpan、S-100、CD117、Dog-1、CD1α、CD34、CD31、ALK、CD3、CD20、CD30、bcl-2、MUM-1、MelanA、HMB45、SMACR、WT-1、PAX5和F8均(-)。结论 HS是一类少见的造血系统恶性肿瘤,原发于小肠更为罕见,肿瘤分化差易误诊,免疫组化有助于HS的诊断。

Objective To investigate the clinical and pathological features, diagnosis and differential diagnosis of histocytic sarcoma(HS) in small intestine. Methods Retrospective analysis of the clinical data, histopathological morphology and immunophentype was conducted in 1 case of HS of small intestine. Results The patient was male, aged 64 years. Clinical presentation was abdominal pain and persistent black lesions. CT showed intussusception of the small intestine but did not except tumors. Histopathological examination demonstrated that the neoplastic cells with abundant and eosinaphilic cytoplasm were diffusely distributed under the intestinal mucosa. Neoplastic cells were large, round to oval,abundant eosinophilic cytoplasm and cytoplasmic vacuoles, with atypia and obvious nucleolus. The tumor cells had an infiltrative behaviour. It was also seen that there were multinucleated inflammatory cells under the background. Immunophentyping showed that the neoplastic cells were positive for Vim, CD68, CD163, Lys and CD10, but negative for CKpan, S-100, CD117, Dog-1, CD1α, CD34, CD31, ALK, CD3, CD20, CD30, bcl-2, MUM-1, MelanA, HMB45, SMA, CR, WT-1, PAX5 and F8. Conclusion HS is an uncommon tumor in the hematopoietic system, and HS in small intestine is much rare. HS with poor morphological differentiation is easily mistaken in diagnosis. Immunophentyping could help the diagnosis of HS.