肺母细胞瘤4例临床分析及文献复习

肺母细胞瘤少见，发病率仅占肺部原发肿瘤的0．25％~0．5％。我院胸外科自1964年4月至2000年2月共收治4例，占同期肺部恶性肿瘤的0．074％（4／5 421），现报告如下并进行有关文献复习。1 材料与方法1．1 一般资料 4例中男女各2例。年龄30~67岁 ，中位年龄48岁。临床症状有咳嗽、血丝痰、胸痛等。病程1个月～6个月，平均3．3个月。肿瘤位于左上叶2例，左下叶1例，右上叶1例。肿瘤直径5．5～16．0cm，平均9．3cm。1．2 诊断方法 胸部X线和CT表现：2例呈单个卵圆形肿块，密度欠均匀，边缘毛糙，有分叶和毛刺，其中1例肺门淋巴结肿大。1例…     

肺母细胞瘤的临床病理特点

肺母细胞瘤是发生于肺的一种少见的恶性混合性肿瘤，其形态结构类似于胚胎期肺组织，本文复习我院三例病例，结合文献，发现主要发生于40岁左右的男性，愈后较差，文献报道最长一例可达24年。需要与之鉴别的病种有：（1）癌肉瘤，（2）来源于支气管腺体的恶性混合瘤，（3）胸膜间皮瘤。     

肺母细胞瘤一例报告及文献复习

肺母细胞瘤又名肺胚瘤或胚胎型癌肉瘤 ,甚为罕见 ,英文文献报告的约 15 0例 ,日文有 35例报告 ,国内报道约 40例。肺母细胞瘤的亚型之一 ,双期型肺母细胞瘤仅占所有原发性肺部肿瘤的 0 .2 5 %～5 %。我科收治一例 ,经化疗达部分缓解后再行手术切除 ,随访至今已生存 18个月。现结合文献报告如下。临床资料　患者男性 ,2 3岁 ,因不明原因出现颜面部及颈部浮肿 ,伴气促、胸痛、咳嗽、咯痰及痰中带血就诊。经胸片及ＣＴ检查示右侧纵隔巨大肿块 (10ｃｍ×14ｃｍ× 2 0ｃｍ ,性质待定 ) ,右肺上叶阻塞性肺不张伴双侧胸腔积液。胸穿抽液示胸水呈乳…     

香菇多糖治疗肺母细胞瘤1例报道及文献复习

肺母细胞瘤(pulmonary blastoma,PB)又称胚胎性癌肉瘤或肺胚瘤,最初由Barrett和Barnard在1945年报道。PB发病率约占肺原发性恶性肿瘤0.25%-0.5%[1],本肿瘤可发生于任何年龄,从2个月至80岁,男女之比约为5∶2。我院与2009年10月17日收治1例肺母细胞瘤患者,现结合此病例复习相关文献做以下报告。     

香菇多糖治疗肺母细胞瘤1例报道及文献复习

肺母细胞瘤（pulmonary blastoma,PB）又称胚胎性癌肉瘤或肺胚瘤,最初由Barrett和Barnard在1945年报道。PB发病率约占肺原发性恶性肿瘤0.25%-0.5%[1],本肿瘤可发生于任何年龄,从2个月至80岁,男女之比约为5∶2。我院与2009年10月17日收治1例肺母细胞瘤患者,现结合此病例复习相关文献做以下报告。     

肺母细胞瘤（附2例报告和文献复习）

2例罕见的肺母细胞瘤均经手术治疗并经病理证实。结合文献讨论肿瘤的病理、鉴别诊断和治疗。X线胸片是重要的诊断方法。首先发现转移病灶是本病特点之一。     

胸膜肺母细胞瘤临床病理分析

背景与目的胸膜肺母细胞瘤是一种罕见的且有其独特临床病理特征的恶性肿瘤,本文旨在探讨胸膜肺母细胞瘤的临床病理学特点及鉴别诊断等。方法收集5例胸膜肺母细胞瘤,复习患者临床资料,进行组织学及免疫组化观察,并结合文献复习分析。结果5例胸膜肺母细胞瘤患者年龄为21个月-47个月,平均为32.8个月;肿瘤主要位于胸腔,4例伴有胸腔积液。组织学观察:I型胸膜肺母细胞瘤1例,呈单纯囊性;II型胸膜肺母细胞瘤2例,呈囊实性,实性区伴横纹肌分化及灶性软骨样结节;III型胸膜肺母细胞瘤2例,完全呈实性,可见间变性未分化肉瘤样成分。免疫组化染色显示肿瘤细胞呈Vimentin(+),部分肿瘤细胞呈Desmin和Myogenin(+),软骨样结节呈S-100(+);PCK、EMA、CD99等均(-)。结论胸膜肺母细胞瘤是一种罕见的主要发生于婴幼儿肺和胸膜的高侵袭性恶性肿瘤,各亚型均有其临床病理学特点。诊断方面应与肺先天性囊性腺瘤样畸形、胚胎性横纹肌肉瘤等良恶性病变鉴别。     

肺炎症性肌纤维母细胞瘤12例临床病理分析

[目的]探讨肺炎症性肌纤维母细胞瘤的病理认识演变和临床特点.[方法]回顾性分析中国医学科学院肿瘤医院胸外科自2005年到2009年间收治的12例肺炎症性肌纤维母细胞瘤病例的临床资料和诊治特点.[结果]本组共12例,占同期肺外科手术的0.23％(12/5 176).其中男性9例,女性3例;平均年龄53.8岁;病灶呈周围型...     

松果体母细胞瘤1例并文献复习

松果体母细胞瘤是罕见的颅内肿瘤,约占松果体实质细胞肿瘤的45％,几乎和松果体细胞瘤各占一半,可见于各年龄段,但多见于20岁以前,好发于儿童,男性略占多数。临床表现多种多样,可表现为颅内压增高,眼神经障碍,精神异常等,临床上与松果体区生殖细胞肿瘤难以鉴别。笔者报道1例松果体母细胞瘤,结合文献对其进行光镜、免疫组化研究,讨论其临床病理特征及诊断与鉴别诊断。     

7例肺母细胞瘤临床分析

0引言肺母细胞瘤( pulmonary blastoma,PB)是一种比较罕见的肺部原发恶性肿瘤,约占所有肺原发恶性肿瘤的0.5%[1].根据发病年龄不同,肺母细胞瘤可分为成人型和儿童型两类.成人型从组织学来源上又分为双向型(biphasic pulmonary blastoma,BPB)和单向型(monophasic pulmonaryblastoma,MPB),前者既含有恶性原始上皮成分,又含有原始间叶成分;后者又叫上皮型或分化好的胎儿型腺癌(well differentiated adenocarcinoma,WDFA),只含有恶性上皮成分[2].     

Classic biphasic pulmonary blastoma: a case report and review of the literature

Pulmonary blastomas are rare malignant tumors, comprising only 0.25-0.5% of all malignant lung neoplasms. Pulmonary blastomas are subdivided in three categories: well-differentiated fetal adenocarcinoma (WDFA), classic biphasic pulmonary blastoma (CBPB) and pleuropulmonary blastoma (PPB), which is currently regarded as a separate entity. The majority of patients with CBPB and WDFA are adults with an average age of 43 years. Tobacco use is identified as a causative agent. Symptomatology varies from asymptomatic (40%) to symptoms of a non-specific pulmonary disease. The most common roentgenologic pattern is a large peripheral nodule. The treatment of choice is surgical excision. The efficacy of adjuvant chemotherapy and radiotherapy is not yet established. The prognosis of pulmonary blastoma is very poor; overall five-year survival is 16%. WDFA appears to have a better prognosis. Adverse prognostic factors are biphasic type, tumor recurrence, metastasis at initial presentation, gross size of the tumor (>5 cm) and lymph node metastasis. On the basis of the available literature, an initial aggressive treatment that includes surgery and, wherever possible, postoperative chemotherapy and radiotherapy could be useful to prolong survival in patients with this rare lung neoplasm. We present a case of classic biphasic pulmonary blastoma in a 77-year old male and review the literature.     

Clinical Analysis of 15 Patients with Adult-Type Pulmonary Blastoma

OBJECTIVE To study the clinical and histopathological characteristics of pulmonary blastoma(PB),and to review the diagnostic criterion and treatment methods.METHODS Clinical and histopathological data from 15 cases of PB in this hospital,from 1990 to 2006,were retrospectively analyzed.RESULTS Surgical procedures were conducted in all 15 patients,including pulmonary lobectomy in 9,lobectomy of the 2 right pulmonary lobes in 2,excision of pulmonary segments in 1,lobectomy and bronchoplasty in 1,lobectomy and angioplasty in 1,and excision of the whole left lung in 1.Of the 15 cases,11 were misdiagnosed as lung cancer,2 were doubted to be a benign tumor before surgery,1 was suspected to be a malignant mesenchymoma,and only 1 was diagnosed as a PB.CONCLUSION Preoperative final diagnosis of PB is very difficult,since the clinical symptoms of the disease are nonspecific.To confirm the diagnosis,comprehensive consideration of the factors,such as the clinical manifestations,morphological characteristics and immunohistochemical tumor markers,should be taken.Surgery is the treatment of choice for PB,and a thorough removal is the key to a cure.PB has a high malignancy and o en recurs and metastasizes,usually with a poor prognosis.     

Retrospective analysis of pulmonary sarcoma and blastoma with review of literatures

To explore the clinical characteristics,diagnosis and treatment of pulmonary sarcoma and blastoma.Methods:Seven cases of pulmonary sarcoma (including 1 case of pulmonary carcinosarcoma,1 case of pulmonary malignant fibroneuroma,1 case of pulmonary malignant fibrous tissue tumor and 1 case of pulmonary blastoma from August 1995 to June 2002 in our hospital)were retrospectively summarized the histological characteristics,clinical features,diagnosis and treatment.Results:Pulmonary sarcoma and blastoma both are rare malignant tumors and can be easily misdiagnosed or mistreated.The main symptoms of both diseases are cough and expectoration.X ray or CT shows node or focus in lung.They both are diagnosed by histological pathology.Because the symptoms of these diseases are not typical,it is difficult for patients to be diagnosed without pathology.Conclusion:These two kinds of diseases are often easy to be misdiagnosed.Early diagnosis and treatment are important to improve the efficiency of treatment.Surgical resection is the best method of treatment ,and it is important to combine with chemotherapy and radiation treatment.     

Unexpected response of a pulmonary blastoma on radiotherapy: a case report and review of the literature

Pulmonary blastoma (PB) is a rare malignant tumor of the lung. Treatment is primarily surgical, although, combination chemotherapy has been reported to result in objective responses in inoperable tumors or after incomplete resections. To our knowledge, this is the first report of a very radiosensitive PB, which showed major tumor reduction after several fractions of radiotherapy without further tumor regression after additional chemotherapy with cisplatin and etoposide. The literature on the treatment of PB is reviewed.     

Solitary brain metastasis from classic biphasic pulmonary blastoma: a case report and review of the literature

BACKGROUND: Classic biphasic pulmonary blastoma (CBPB) is a rare and aggressive primary malignancy, brain metastases of this type of tumor are even rarer. CASE REPORT: A 51-year-old male patient with a solitary cerebral metastasis, diagnosed ten months after left pneumonectomy for a CBPB, was treated by surgery and accelerated hypofractionated radiotherapy. RESULTS: The patient died 15 months after partial removal of the brain metastasis. Literature review revealed only 4 cases of solitary brain metastases from this type of malignancy. The present case is the second one reported with a combined treatment of surgery and radiotherapy resulting in the longest survival. CONCLUSION: The best treatment for CBPB is difficult to determine because of the small number of cases, however, the combination of surgery with radiotherapy seems to be effective. The effectiveness of chemotherapy has not been ascertained.     

Pulmonary blastoma. Case report and literature review of chemotherapy experience

Pulmonary blastoma is a rare primary lung neoplasm, occurring in both children and adults, which is pathologically, clinically, and prognostically distinct from other lung tumors. Usually it has been treated with surgery, but both chemotherapy and radiotherapy have been used for metastatic disease, and in the adjuvant setting. A patient is described who presented with metastatic pulmonary blastoma. Treatment with cyclophosphamide, vincristine, doxorubicin, and dactinomycin resulted in an objective response as judged by standard criteria. The literature is reviewed for other experience with chemotherapy in this rare lung tumor. This four-drug combination appears to show promise for tumor response, and is deserving of further trial.     

Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood

The authors studied 11 pediatric intrathoracic neoplasms that share clinicopathologic features and constitute a specific tumor in children. These neoplasms were intrapulmonary, mediastinal, or pleural-based masses. A common histologic feature was the presence of small, primitive cells with blastematous qualities separated by an uncommitted stroma. Focal rhabdomyosarcomatous, chondrosarcomatous, and liposarcomatous differentiation was observed. Epithelial components had bland cytologic features and probably represented entrapped benign epithelium and/or mesothelium. The prognosis for these patients was grave; seven patients died of their disease 5 months to 2 years after diagnosis. Two patients have survived disease-free for 10 and 12 years after diagnosis. Two recent cases are alive 14 and 32 months after diagnosis. This neoplasm constitutes a distinct entity which has been reported in the literature as pulmonary blastoma in children. It differs from pulmonary blastoma in adults because of its variable anatomic location, primitive embryonic-like blastema and stroma, absence of a carcinomatous component, and potential for sarcomatous differentiation. The designation of pleuropulmonary blastoma is suggested by the authors for these intrathoracic neoplasms of childhood rather than pulmonary blastoma for histogenetic and anatomic reasons. The clinicopathologic features, immunophenotypic and ultrastructural characteristics, possible histogenesis, and differential diagnosis of these neoplasms from other thoracopulmonary tumors in children serve as the basis for this report.     

成人型肺母细胞瘤五例报告及文献复习

背景与目的　肺母细胞瘤是一种少见的肺部原发恶性肿瘤,分为成人型肺母细胞瘤和儿童胸膜肺母细胞瘤。本文主要讨论成人型肺母细胞瘤的临床特点、诊断和治疗。方法　回顾我院从1964 年4 月至2004年3月收治的5例成人型肺母细胞瘤的临床资料,并复习有关国内外文献。结果　5 例中男3 例,女2例;发病年龄22~70岁;以咳嗽、咳血丝痰为主要症状,次为胸闷、胸痛;影像学主要表现为肺部单发实性肿块,纤维支气管镜检查及痰细胞学检查均没有发现肿瘤细胞。3 例行肺叶切除加肺门纵隔淋巴结清扫术,1例行左全肺切除加肺门纵隔淋巴结清扫术,1例行肺楔形切除术并行术后化疗。到随访结束,3 例死亡,2 例生存,生存期最短6个月,最长11年。结论　肺母细胞瘤术前难明确诊断。治疗以肺叶切除加纵隔淋巴结清扫术为主,对放疗及化疗不敏感,预后差别较大。     

肺母细胞瘤的研究进展

肺母细胞瘤是一种罕见肺部原发恶性肿瘤,分为成人型肺母细胞瘤和儿童胸膜肺母细胞瘤.肺母细胞瘤的临床症状及影像学检查无特异性,容易误诊,术前诊断困难,术后病理需结合形态学和免疫组化综合考虑.本病首选手术治疗,手术方式以肺叶切除加纵隔淋巴结清扫术为主.预后与病理类型、分期及肿瘤部位等有关,总体预后差.