CAJ | 학술논문

目的分析体表转移性神经母细胞瘤(NB)的临床、病理形态学特点、诊断与鉴别诊断及预后.方法回顾分析本院2005年至2011年间14例诊断为NB体表转移病例的临床、病理学资料及随访资料.男8例,女6例.发病年龄5个月～6岁10个月,平均发病年龄33个月,年龄＜1.5岁3例,1.5～3岁4例,＞3岁7例.肿瘤原发部位:后腹膜8例,肾上腺4例,盆腔1例,纵膈1例.体表转移部位:体表淋巴结转移共11例(78.6％),其中颈部淋巴结9例、腹股沟淋巴结2例;肩背部皮下1例,臀部皮下1例;阴囊1例.组织学分型:未分化型3例,分化差型9例,分化型2例.未分化型与分化差型共12例(85.7％).结果 14例NB体表转移患儿,随访时间平均13个月,9例(64.3％)放弃治疗后死亡,3例化疗后死亡,2例(14.3％)3年无瘤生存.免疫组化染色结果:14例NB体表转移的肿瘤细胞Syn与CgA均弥漫性强阳性表达,NSE除1例未分化型NB外其余均阳性表达.肿瘤细胞对Vim、LCA、CD99、DES、S-100等抗体均阴性表达.肿瘤组织中神经鞘膜细胞阳性表达S-100.结论 NB转移至体表多处部位以淋巴结转移最多见,以未分化型及分化差型为主要类型,预后较差.免疫组化有助于诊断与鉴别诊断.
목적 분석체표전이성신경모세포류(NB)적림상、병리형태학특점、진단여감별진단급예후.방법 회고분석본원2005년지2011년간14례진단위NB체표전이병례적림상、병이학자료급수방자료.남8례,녀6례.발병년령5개월～6세10개월,평균발병년령33개월,년령＜1.5세3례,1.5～3세4례,＞3세7례.종류원발부위:후복막8례,신상선4례,분강1례,종격1례.체표전이부위:체표림파결전이공11례(78.6％),기중경부림파결9례、복고구림파결2례;견배부피하1례,둔부피하1례;음낭1례.조직학분형:미분화형3례,분화차형9례,분화형2례.미분화형여분화차형공12례(85.7％).결과 14례NB체표전이환인,수방시간평균13개월,9례(64.3％)방기치료후사망,3례화료후사망,2례(14.3％)3년무류생존.면역조화염색결과:14례NB체표전이적종류세포Syn여CgA균미만성강양성표체,NSE제1례미분화형NB외기여균양성표체.종류세포대Vim、LCA、CD99、DES、S-100등항체균음성표체.종류조직중신경초막세포양성표체S-100.결론 NB전이지체표다처부위이림파결전이최다견,이미분화형급분화차형위주요류형,예후교차.면역조화유조우진단여감별진단.
Objective To analyze the clinicopathologic features,diagnosis,differential diagnosis and prognosis of body surface metastatic neuroblastoma (NB).Methods A total of 14 cases with a preliminary diagnosis of body surface metastatic neuroblastoma at our hospital from 2005 to 2011 were collected.And their clinicopathologic findings,immunohistochemical results and follow-up data were recorded and analyzed.Results There were 8 males and 6 females with an average age of 33 (5-82) months.And 7 cases (50％) were above 3 years.The metastatic sites were lymph node (n =11),neck (n =9) and inguinal (n =2).The subcutaneous metastasis was located in shoulder (n =1),gluteal region (n =1) and scrotum (n =1).Histological type were undifferentiated (n =3),poor differentiation (n =9) and differentiated (n =2).The average follow-up time was 13 months and the 3-year event-free survival rate 14.3％ (2/14).Nine cases (64.3％) died of discontinued therapy and another 3 cases died after chemotherapy.Immunohistochemistry:In NB tumor cells,Syn and CgA were strong positive expressed.Neuron-specific enolase (NSE) was positive in all cases except for one undifferentiated NB.Schwann cells in tumor tissues were positive for S-100 but all tumor cells negative for Vim,LCA,CD99,DES or S-100.Conclusions Most types of body surface metastasis of NB are undifferentiated and poorly differentiated.And the prognosis is poor.Immunohistochemistry aids the diagnosis and differential diagnosis.