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目的:探讨鼻腔嗅神经母细胞瘤的临床病理学特征,提高本病的诊治水平。方法:回顾分析1965~1997年间收治的4例经病理证实为本病患者的临床与病理资料,并对其中1例进行光镜、电镜和免疫组化染色观察。结果:4例患者术后和/或放疗后7年内部复发,其中3例患者在半年至7年内死亡,1例存活5年。光镜下瘤细胞大小较一致呈放射状,有假菊花团样结构。电镜下瘤细胞分明暗两类,胞质内偶见神经内分泌颗粒及各种细胞器。免疫组化染色见瘤细胞神经特异性烯醇酶(NSE)及S100蛋白呈阳性反应,角蛋白阴性。结论:(1)本病易复发且易向颅内转移,有侵袭性,晚期患者预后差,死亡率高;(2)电镜及免疫组化染色检查在鉴别诊断中有重要意义;(3)本病应早期进行根治性手术并辅助放射治疗。
Objective: To investigate the clinicopathological features of nasal olfactory neuroblastoma and to improve the diagnosis and treatment of this disease. Methods: The clinical and pathological data of 4 pathologically confirmed patients admitted to our hospital from 1965 to 1997 were retrospectively analyzed. One of them was observed by light microscope, electron microscope and immunohistochemistry. RESULTS: Four patients relapsed within 7 years after surgery and / or radiotherapy. Three of these patients died within six months to seven years and one survived five years. Under the light microscope, the size of the tumor cells was more consistent and radiate, with the false-like daisy-like structure. Under electron microscopy tumor cells were dark and dark, the cytoplasm occasionally neuroendocrine granules and various organelles. Immunohistochemical staining of neoplastic neuron-specific enolase (NSE) and S-100 protein positive reaction, keratin-negative. Conclusion: (1) The disease is easy to recurrence and easy to intracranial metastasis, invasive, advanced patients with poor prognosis and high mortality; (2) electron microscopy and immunohistochemical staining in the differential diagnosis of great significance; (3) The disease should be an early radical surgery and adjuvant radiotherapy.