本文選題：促結(jié)締組織增生性小圓細胞腫瘤　切入點：鼻腔鼻竇　出處：《復旦學報(醫(yī)學版)》2017年01期 　論文類型：期刊論文

【摘要】：目的探討鼻腔鼻竇促結(jié)締組織增生性小圓細胞腫瘤(desmoplastic small round cell tumor,DSRCT)的臨床病理特征、免疫表型、分子遺傳學改變及其診斷與鑒別診斷。方法對復旦大學附屬眼耳鼻喉科醫(yī)院新發(fā)1例及文獻報道的另2例原發(fā)于鼻腔鼻竇的DSRCT進行總結(jié),分析其臨床數(shù)據(jù)、病理學形態(tài)表現(xiàn)、免疫表型和分子病理改變。結(jié)果 3例均為原發(fā)于鼻腔鼻竇的DSRCT,男性1例,女性2例,平均年齡43歲(21~61歲),部位分別為上頜竇、蝶竇和篩竇,并不同程度地侵犯周圍組織。臨床表現(xiàn)為鼻塞、鼻出血、通氣不暢。3例組織學形態(tài)表現(xiàn)均與經(jīng)典部位的DSRCT相似,腫瘤由外形不規(guī)則的核深染小圓細胞組成,被增生的纖維結(jié)締組織分隔成大小不一的巢團,細胞分化差,核分裂像易見,并伴不同程度的壞死和侵襲性生長。其中本院病例部分腫瘤細胞呈上皮樣、橫紋肌樣或漿細胞樣,局部形成假菊形團樣結(jié)構(gòu)。免疫組化染色結(jié)果顯示瘤細胞均表達上皮、間葉和神經(jīng)源性標記物。3例分別經(jīng)FISH、RT-PCR及Southern blot檢測證實存在EWS-WT1基因融合。此外,本院病例行電鏡檢查顯示,瘤細胞具有大量樹突狀突起和細胞器,可見核旁豐富的中間絲。3例均行手術(shù)切除,并結(jié)合輔助性放化療,其中2例文獻報道的患者無瘤生存期超過2.5年,1例本院患者于術(shù)后4個月復發(fā),1年后死亡。結(jié)論 DSRCT是一種少見的高度惡性腫瘤,發(fā)生在鼻腔鼻竇處更為罕見且預后差,充分認識其病理學特點并了解其發(fā)生的罕見部位,有助于避免漏診和誤診。
[Abstract]:Objective to investigate the clinicopathological features and immunophenotype of desmoplastic small round cell tumor DSRCTs. Methods the clinical data and pathological features of 1 new case of DSRCT in the nasal cavity and paranasal sinuses and 2 other cases reported in the literature were summarized, and the clinical data and pathological features of the patients were analyzed, including the molecular genetic changes, the diagnosis and differential diagnosis of the disease, and the diagnosis and differential diagnosis of the disease in the hospital of Otolaryngology, affiliated to Fudan University. Results all 3 cases were primary DSRCTs of nasal cavity and paranasal sinus, male 1 case, female 2 cases, mean age 43 years old 21 61 years old, maxillary sinus, sphenoid sinus and ethmoid sinus, respectively. The clinical manifestations were nasal congestion, epistaxis, and unobstructed ventilation. The histologic appearance of the tumor was similar to that of the classical DSRCT, and the tumor was composed of small round cells with irregular nuclear staining. Divided by proliferative fibrous connective tissue into nests of different sizes, the cells were poorly differentiated, mitotic images were easily seen and accompanied by varying degrees of necrosis and invasive growth. Some of the tumor cells in our hospital were epithelioid, rhabdomyoid or plasmacytoid. The results of immunohistochemical staining showed that all the tumor cells expressed epithelium, the mesenchymal and neurogenic markers in 3 cases confirmed the existence of EWS-WT1 gene fusion by fish RT-PCR and Southern blot. A large number of dendritic processes and organelles were found in the tumor cells. 3 cases with abundant intermediate filaments around the nucleus were surgically removed and combined with adjuvant radiotherapy and chemotherapy. Two of the cases reported in the literature had a tumor-free survival of more than 2.5 years. One patient recurred 4 months after operation and died one year later. Conclusion DSRCT is a rare and highly malignant tumor, which is more rare in the nasal cavity and paranasal sinus and has a poor prognosis. It is helpful to avoid missed diagnosis and misdiagnosis by fully understanding the pathological characteristics and the rare site of its occurrence.
【作者單位】： 復旦大學附屬眼耳鼻喉科醫(yī)院病理科;
【分類號】：R739.62

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