鼻腔鼻窦原发孤立性纤维性肿瘤的临床病理观察

Clinicopathological observation of solitary fibrous tumors in nasal cavity and paranasal sinus

为探讨原发鼻腔鼻窦内的孤立性纤维性肿瘤(solitary fibrous tumors,SFT)的临床特点、影像学特点、病理表现、治疗及预后,进一步明确鼻腔鼻窦SFT的诊断要点,本文分析4例我院收治的原发在鼻腔鼻窦内的SFT患者的临床资料,观察其组织学形态及免疫表型特征,并复习相关文献。4例患者鼻腔内窥镜术后标本病理检查结果示:肿瘤位于黏膜下,具有假包膜,温和的梭形细胞杂乱分布在胶原化的间质,可见细胞稀疏区及细胞致密区,肿瘤围绕明显的分支状血管,呈血管外皮瘤样排列。胞质轻度嗜酸性,细胞核呈卵圆形,染色质细腻,核仁不明显。免疫表型结果示:肿瘤细胞弥漫阳性表达STAT6(细胞核)和CD34,同时也阳性表达CD99和Bcl-2,不表达Desmin,S-100,EMA,CK。Ki-67增殖指数约1%~5%。原发在鼻腔鼻窦内的SFT是一种交界或具有低度恶性潜能的软组织肿瘤,临床上非常罕见,5%~10%的病例具有复发的可能性,因此,预后可能与肿瘤是否完整切除密切相关。

To investigate the clinical features,imaging features,pathological manifestations,treatment and prognosis of solitary fibrous tumors(SFT)originate in nasal cavity and paranasal sinus,and to further clarify the diagnostic points of SFT,the clinical data of 4 patients with SFT in the nasal cavity and paranasal sinus from our hospital were analyzed.The histological and immunophenotypic features of these tumors were observed and the related literatures were reviewed.Pathological examination of the specimens of four patients after endoscopic sinus surgery showed that under the microscope,tumors were submucosal and pseudoencapsulated.Bland plump spindle cells dispersed within a collagenous stroma,haphazard arrangement,hypercellular and hypocellular sclerotic foci.There was a prominent branching vasculature around the tumor cells arranged as hemangiopericytoma-like.Tumor cells had scant eosinophilic cytoplasm,ovoid nuclei with fine chromatin,and inconspicuous nucleoli.Immunohistochemically,tumors were diffuse positive for STAT6(nuclear)and CD34,also positive for BCL2 and CD99,but non-reactive with desmin,S100,EMA and CK.The proliferation index of Ki-67 was about 1–5%.SFT in nasal cavity and paranasal sinus is a kind of borderline/low-grade malignant soft tissue tumor,which is extremely rare.About 5%to 10%of all SFTs have shown recurrence.Therefore,the prognosis may be closely related to the completeness of the surgical resection.