【作者】 雷文斌； 苏振忠； 文卫平； 史剑波； 廖冰； 文剑明； 许庚；

【Author】 Otorhinolaryngology Hospital, Otorhinolaryngology Institute, Department of pathology, The First Affiliated Hospital, SUN Yat- sen University, Guangzhou 510080,China,

【机构】 中山大学附属第一医院耳鼻咽喉科医院耳鼻咽喉科学研究所； 中山大学附属第一医院病理教研室；

【摘要】 目的:探讨鼻颅底小圆细胞恶性肿瘤的特征和准确快速的诊断方法。方法:回顾性复习本院既往10年组织病理学特点为小圆细胞的鼻颅底恶性肿瘤122例,比较它们的临床特点,组织学特征和免疫组化结果。结果:122例患者,临床上多表现为鼻塞,头痛,涕中带血,复视,视力下降伴体重减轻等。体检发现多数病例单侧鼻腔鼻窦有粗糙易出血的肿物。组织学上是分化差的小圆细胞;免疫组化检测,多数病例都在检测5—6个标记物后作出诊断,个别甚至检测20多个标记物才确诊;本组恶性淋巴瘤43例,LCA阳性达38／ 43,L26(18／43),UCHL-1(14／43),CD3(5／43),CD56 (6／43),CD30和CD79(4／43)表达阳性;低、未分化癌35 例,均有CK表达阳性;原始外胚叶恶性肿瘤10例,NSE(9／ 10)阳性,其它神经标记物Syn(5／10)、S—100(4／10), Vimintin(4／10)CgA(2／10)阳性;嗅神经母细胞瘤15 例,NSE阳性(13／15),其它神经标记物S-100(10／15)CgA (8／10),Syn、GFAP、CK和PAS分别(7／15)阳性。胚胎性横纹肌肉瘤7例,其间叶源性和肌源性标记物Vimintin(7／ 7),Desmin(6／7)、HHF35(3／7)、肌球蛋白(Myosin) 和肌红蛋白(Myoglobin)(2／7)阳性浆细胞瘤4例均有LCA、λ或κ轻链及CD56表达阳性;尤文氏(Ewing)肉瘤2例, 均有CD99和Vimintin表达阳性;恶性黑色素瘤2例其 HMB45和S-100均表达阳性;肝细胞癌颅底转移2例, 其Hepatocyte和AFP表达阳性,间叶性软骨肉瘤3例,免疫组化检测无特异性。结论:本组病例的临床症状、体征及影象学改变对肿物的恶性生物学特性有一定的提示;组织学特征可进一步确定病变为小圆细胞恶性肿瘤,但难以单凭此作出具体诊断;各种标记物的免疫组化检测有助于确定病变的组织来源,是确诊关键之一;多学科合作,综合临床资料和组织学特征,并选择合适的标记物进行免疫组化检测,不但能明确诊断,还可有效地减少诊断延误,使病人得到及时和准确的治疗。更多还原

【Abstract】 Objective: To investigate a quick and accurate pathological diagnosis way of small round cell tumor of nasal sinus and bases of skull. Methods: 122 cases of nasal sinus and bases of skull small round cell tumors of our hospital in the past ten year were analyzed retrospectively. Clinical, histological and immunohis-tochemical characters of these cases were compared. Results: 122 cases of patients, usually complained of nasal obstruction, headache, diplopia, nasal mucus with bleeding, losed their eyes and weight loss. A lots small round cells were found in these tumors in histological pathology. Most of these cases were finally diagnosed after immunohistochemical examination of at less 5-6 cell, tissue or tumor markers ( antibody ). Some of them were examined more than 20 markers. Malignant lymphoma was in 43 cases, markers LCA (38/43), L26 (18/43 ), UCHL-1 (14/43), CD3 ( 5/43 ), CD56 (6/43 ), CD3 and CD79 (4/ 43 ) were found positive in these cases. Poorly differentiate squamous carcinoma was in 35 cases, marker CK was found positive in all of the cases. Peripheral primitive neu-roectodermal tumor (PNET) was in 10 cases, markers NSE (9/10 ), Syn (5/10), S-100 (4/10 ), Vimintin( 4/10 ) and CgA( 2/10 )were found positive. Olfactory neuroblastoma was in 15 cases, markers NSE(13/15), S-100(10/15), Cga (8/15), Syn(7/15), GFAP(7/15), CK,(7/15) and PAS(7/15) were found positive .Embryonal rhabdomyosarcoma was in 7 cases, markers Vimintin (7/7 ), Desmin ( 6/7 ),HHF35 (3/7 ),Myosin(2/7) and Myoglobin(2/7) were found positive. Plasmacytoma was in 4 cases, λ, κ light chain , LCA and CD56 were found positive in all of the cases. Ewing sarcoma was in 2 cases, both CD99 and Vimintin were found positive. Chromoma was in 2 cases, both HMB45 and S-100 were found positive. Nasal sinus or bases of skull found hepatoma metastasis was in 2 cases, both Hepatocyte and AFP were found positive. Chondrosarcoma was in 3 cases , no special immunohistochemical result was found. Conclusion: Clinical symptom, physical signs and radiological finding could supply some malignant evidences of these tumors. Histological examination could make certain that they were small round cell tumors, but final diagnosis was still hard to make on this, Immunohistochemical examination of various markers could tell the specimen’ s tissue origin characters, it was one of the key for final diagnosis. Collaboration of various departments, aggregate analysis of clinical and histological characters, suitable selection of markers for immuno, could not only made for final diagnosis, but also avoid dally over of diagnosis. These patients would get appropriate treatments in time.更多还原