胃血管球瘤5例临床病理学特征与鉴别诊断

Clinical and pathological features of gastric glomus tumor

目的探讨胃血管球瘤的临床病理学、免疫组化和生物学行为特点。方法观察5例胃血管球瘤的临床表现,病理形态特征及免疫组化表型,并复习文献,探讨其生物学行为特征。免疫组化采用SP法。检测用抗体包括vimentin、actin、a-SMA、MSA、CD117、CD34、CK、Syn、CgA、LCA、desmin、CD99、S-100、EMA和Ki-67。结果 5例患者中,男性1例,女性4例,平均年龄42岁(34～65岁)。常见症状为上腹部疼痛。肿物直径为0.8～5 cm(平均为2.4 cm)。巨检:肿物界清、无包膜,切面灰白色,质实。镜检:肿瘤组织呈结节分叶状,被平滑肌细胞分隔包绕。肿瘤细胞大小一致,呈圆形或卵圆形,围绕薄壁的血管或血窦周围排列,呈血管外皮瘤样或实性巢团状,瘤细胞核圆形,可见核仁,未见核异型性,核分裂象罕见(0～3个/50 HPF),未见病理核分裂象。1例瘤体直径5 cm者瘤细胞较密集,有局灶异型,核分裂象(1个/50 HPF),诊断为恶性潜能未定肿瘤。免疫组化:vimentin、actin、a-SMA和MSA均(+),余CD117、CD34、CK、Syn、CgA、LCA、desmin、CD99、S-100和EMA均(-),Ki-67阳性率<2%。5例均行胃肿块切除。随访8个月至5年,未见复发或转移。结论胃血管球瘤绝大多数为良性肿瘤,与软组织血管球瘤比较,具有自身病理形态学及生物学行为的特征。

Objective To investigate the clinicopathological,immune phenotype and biological behavior features of gastric glomus tumor.Methods Clinical manifestations,pathological features and immunohistochemical characteristics were analyzed in 5 cases of gastric glomus tumor,with reviewe of relevant literature focused on its biological behavior features.Immunohistochemistry was used with SP method,with antibodies including vimentin,actin,a-SMA,MSA,CD117,CD34,CK,SYN,CGA,LCA,Desmin,CD99,S-100,EMA and Ki-67.Results One male and four females were present in five patients,with average age 42 years（34~65 years old）,upper abdominal pain was the common symptoms,tumor maximum diameter was 5.0 cm（average diameter of 2.4 cm） and no tumor recurrence or metastasis of postoperative follow-up.Grossly,the tumor was with no capsule,gray cut surface and solid quality.Microscopically tumor tissue was nodular lobulated,separated and enveloped by smooth muscle cells.Tumor cells were round or oval with uniform size,with arrangement around the thin-walled blood vessels or sinuses in a way of hemangiopericytoma-like or solid nests.Tumor nuclei were round with visible nucleoli,no nuclear atypia,rare mitoses（0~3/50 HPF）,without pathological mitoses.One case that tumor diameter was 5.0 cm and tumor cells were dense,focal profiled and mitotic activity（1 / 50 HPF） was diagnosed undetermined malignant potential tumor.The total 5 cases of gastric glomus tumor with gastric mass were found no recurrence or metastasis under follow-up from 8 months to 5 years.The immunohistochemical staining was showed positive in vimentin,actin,a-SMA and MSA,and negative in CD117,CD34,CK,Syn,CgA,LCA,Desmin,CD99,S-100,EMA and Ki-67 index ＆lt;2%.Conclusion Gastric glomus tumor is a rare,almost benign tumor,which has its own pathological and biological behavior characteristics compared with soft tissue glomus tumor.