上消化道颗粒细胞肿瘤10例临床病理学分析

Granular cell tumors of upper digestive tract:a clinicopathological study of 10 cases

目的探讨上消化道颗粒细胞瘤的临床病理学特点以及治疗和预后。方法观察10例上消化道颗粒细胞瘤的临床、组织形态学以及免疫组织化学等特征。结果患者平均年龄40.5岁,男女比例为7∶3。9例上消化道颗粒细胞肿瘤位于食管,1例位于胃。肿块多位于黏膜层至黏膜下层。组织学上,肿瘤细胞呈紧密的巢状或片状,多呈浸润性生长,但常无坏死。细胞呈圆形、多边形或梭形,细胞质丰富且含有多量呈PAS-D阳性的嗜双色性颗粒状物质,细胞核小、圆而居中,核分裂象少见。免疫谱示肿瘤细胞均呈弥漫强阳性表达nestin和S-100,多呈CD68差异性阳性表达,而CK、CD117、CD34、desmin和SMA均阴性。经随访12～54个月,10例肿瘤均未见复发和转移。结论上消化道颗粒细胞肿瘤少见,但特征性的组织学和免疫组织化学特征有助于诊断,多行内窥镜下或手术切除治疗,常具有良性的临床经过。

Purpose To investigate the clinicopathological features,management and prognosis of granular cell tumors（GCTs） in upper digestive tract.Methods The clinical data,histological features and immunohistochemical profiles were studied in 10 cases of granular cell tumors in upper digestive tract.Results Nine cases with GCTs of the upper digestive tract were found in esophagus,and 1 case in stomach.The averge age of the patients was 40.5 years old.The male/female ratio was 7 ∶3.The tumors typically involved the mucosa,lamina propria and submucosa.Histologically,the tumor composed of compact sheets or nests of plump,round or polygonal cells with abundant lightly amphophilic or eosinophilic but strikingly granular cytoplasm and uniformly small,round,and pyknotic central nuclei.The cytoplasm was diffusely periodic acid-Schiff positivity,even after diastase digestion.Local tumor infiltration and areas of spindling present but mitoses were rare to absent,and necrosis absent.Immunohistochemically,GCTs were immunoreactivity to nestin,S-100 proteins in all cases and CD68 with varied degree in most cases,but negative for CK,CD117,CD34,desmin and SMA.Recurrence or metastasis was not detected in all 10 cases during 12 to 54 months of follow-up.Conclusions GCTs in the upper digestive tract were rare,showed characteristic histology and immunohistochemistry,and often possessed benign behavior after endoscopic mucosal dissection or surgical resection.