肢体副神经节瘤的临床病理特征和鉴别诊断(附病例报告)

目的探讨肢体这一罕见部位副神经节瘤的病理诊断和鉴别诊断。方法对1例肢体副神经节瘤进行光镜观察和免疫组化染色,并复习文献资料。结果副神经节瘤生长缓慢,大多包膜完整,无出血坏死;镜下肿瘤细胞呈腺泡状或细胞球状排列,血窦丰富,分裂象少或无;主细胞恒定表达NSE、CgA等神经内分泌标记物,支持细胞表达S-100、GFAP,阳性信号围绕细胞巢周围较有特征性,但有时无表达,Ki-67增殖活性低。结论副神经节瘤发生于肢体非常罕见,极易与众多恶性肿瘤混淆,综合分析其临床病理特征,可有效避免误诊。

The clinicopathologic features and differential diagnosis of extremity paraganglioma: a case report

Objective To investigate the pathologic diagnosis and differential diagnosis of extremity paraganglioma.Method One case of extremity paraganglioma was studied with conventional pathological and immunohistochemical stains,and its clinical and pathological findings were analyzed with review of the literature.Result Extremity paraganglioma had a long history of slow growth.On gross examination,the tumor appeared well-encapsulted without necrosis and hemorrhage.Histologic features consisted of alveolar architecture or Zellballen pattern,prominent sinusoids,no or low mitotic activity.Chief cells were positive for neuroendocrine markers,such as NSE and CgA.A chraracteristic immunostain for S-100 and GFAP,but sometimes negative,demonstrating distinctive sustentacular cells,could help to arrive in diagnosis.Ki-67 index showed low level.Conclusion Because of the rarity of lesion site,extremity paraganglioma is often confused with other malignant tumors.The correct diagnosis could be made through careful analysing the characteristics of clinical pathology.