肺部少见的原发恶性肿瘤——附19例报告

目的:探讨肺部少见原发性恶性肿瘤的临床特点、诊断和治疗方法。方法:总结我院1987年~2006年的肺少见原发性恶性肿瘤共19例临床资料结合文献进行分析。结果:19例病人中肺平滑肌肉瘤3例,肺纤维肉瘤3例,肺软骨肉瘤1例,肺母细胞瘤2例,肺癌肉瘤1例,肺恶性淋巴瘤3例,支气管乳头状瘤恶变2例,肺恶性神经鞘瘤1例,肺恶性血管外皮瘤2例,肺恶性黑色素瘤1例。全组病人均经手术切除病变肺组织,其中肺叶切除17例,肺段切除1例,全肺切除1例。无手术及术后住院期死亡,无术后严重并发症。结论:手术是肺部少见原发性恶性肿瘤主要的诊断和治疗手段。     

肺癌肉瘤13例临床分析

目的分析肺癌肉瘤的临床表现及特点,探讨肺癌肉瘤的预后因素及治疗方式。方法对13例肺癌肉瘤患者的临床资料,采用SPSS 18.0统计学软件进行数据分析,应用Kaplan-Meier法计算其生存率(overall survival rate,OS),并进行单因素分析。结果 13例肺癌肉瘤中,癌成分为鳞癌7例、腺癌4例(其中1例合并小细胞癌)、腺鳞癌1例、未分化癌1例。淋巴结转移以癌成分为主。单因素分析结果显示,肺癌肉瘤癌成分(P=0.047)及手术方式(P=0.019)是影响其预后的因素。结论肺癌肉瘤是肺肉瘤样癌的1种亚型,包含癌成分及肉瘤成分,其中癌成分为鳞癌者预后好于非鳞癌者(P=0.047);根治性手术是治疗肺癌肉瘤的主要方法,肺癌肉瘤术后易发生远处转移,发生远处转移后采用综合治疗,有利于提高患者远期生存率。     

A case of rapidly growing pulmonary carcinosarcoma

We report herein a case of rapidly growing pulmonary carcinosarcoma, a rare and highly malignant lung neoplasm characterized by a biphasic histopathological pattern consisting of both epithelial and sarcomatous components, and we also summarize the clinical features of this entity based on previously reported cases. A 65-year-old man was referred for further examination of a lung tumor after a routine chest X-ray (CXR) showed a tumor shadow in the right upper lung zone. Chest computed tomography (CT) found a 2.0 cm pulmonary mass with suspected chest wall invasion in the right upper lobe, although cytological evidence of malignancy could not be obtained despite repeated preoperative bronchoscopy. The tumor grew rapidly, indicating the possibility of lung cancer. A right upper lobectomy with chest wall excision was performed. The postoperative definitive diagnosis was carcinosarcoma consisting of adenocarcinoma and chondrosarcoma. The pathological stage was p-T3N0M0. The patient subsequently received adjuvant chemotherapy with cisplatin and vinorelbine. Routine follow-up chest CT 7 months after the surgery showed pleural dissemination. Consequently he underwent radiotherapy, but the disseminated tumors enlarged further while he received this treatment. The patient is receiving best supportive care at present. Findings based on previously reported cases and our case suggest that early surgical intervention and combined therapeutic strategy are the most important aspects of treatment for pulmonary carcinosarcoma.     

Malignant mixed tumor

The term "malignant mixed tumor" is usually synonymous with "carcinoma in pleomorphic adenoma," a secondary carcinoma developing in pre-existing pleomorphic adenoma. However, it sometimes indicates a group of tumors consisting of carcinoma in pleomorphic adenoma, carcinosarcoma (true malignant mixed tumor) and metastasizing benign mixed tumor, the latter 2 being the most infrequent. According to the data of the Japanese committee on TNM classification for salivary gland carcinomas, carcinoma in pleomorphic adenoma accounted for about 10% of all salivary gland carcinomas, both in the parotid and submandibular glands. The main type of carcinomas arising in pleomorphic adenoma were undifferentiated carcinoma, adenocarcinoma and squamous cell carcinoma. Crude 5- and 10-year survival rates were 54.7% and 42.7%, respectively. Invasive carcinomas and carcinomas of high grade malignancy carried worse prognoses. The treatment of choice for carcinoma in pleomorphic adenoma has consisted of en-bloc excision with wide margin. Invasive growth, facial nerve involvement, lymph node metastasis or high-grade malignant tumor are grounds for postoperative radiation therapy. The role of chemotherapy has not yet been well established.     

The first case of lung carcinosarcoma harboring in-frame deletions at exon19 in the EGFR gene

Mutations of the epidermal growth factor receptor (EGFR) gene play a critical role in carcinogenesis of lung cancer, particularly adenocarcinoma. However, to the best of our knowledge, no mutations of the EGFR in patients with lung carcinosarcoma have been identified. We herein report the case of a 61-year-old female referred for a detailed examination of a left pulmonary mass shadow. Although bronchoscopy was performed, it failed to lead to a diagnosis, and video-assisted thoracoscopic surgery was therefore carried out to diagnose the tumor. The pathology revealed biphasic features consisting of both adenocarcinoma and chondrosarcoma. Intriguingly, both the adenocarcinoma and chondrosarcoma components were proven to harbor an exon19 deletion in the EGFR gene. Although carcinosarcoma is a rare malignancy of the lungs, genetic analyses of oncogenic drivers, such as the EGFR gene, should be conducted.     

So-called gastric carcinosarcoma--a case of chondrosarcomatous undifferentiated in the metastatic foci

A case of so-called gastric carcinosaroma in a 70-year-old man was presented. The primary tumor measuring 5.6 X 5.4 cm was located in the gastric antrum and had the appearance of Borrmann 2 type. A massive metastatic tumor was found in the pyloric lymph nodes. Histologically, the primary tumor was predominantly composed of papillary adenocarcinoma invading to the muscle layer with lymphatic and vascular involvements. Atypical spindle cells occasionally intervened between carcinoma nests. The metastatic tumor consisted of mixed papillary adenocarcinoma and well differentiated chondrosarcoma with transitional areas by atypical spindle cells. Therefore, it was suggested that sarcomatous components in the present case were derived from carcinoma cells by undifferentiated. Three cases of gastric carcinosarcoma reported in Japan were reviewed, and their histogenesis was discussed as compared with the present case.     

Differential macromolecular leakage from the vasculature of tumors

Tumor-induced neovascularization is essential for invasion, metastases, and exponential growth of solid tumors. The authors studied the differences in macromolecular leakage from the neovasculature of a fast-growing, early-metastasizing tumor, the Walker 256 carcinosarcoma, and a slow-growing, nonmetastasizing tumor, a rat chondrosarcoma. A 1-mm3 piece of the Walker 256 carcinoma or the chondrosarcoma was implanted in the cremaster muscle of rats. Five days after surgery the cremaster muscle with the implanted tumor was placed in a special bath containing Krebs solution such that the circulation and nerves from the animal to the cremaster were intact. Fluorescein isothiocyanate-labeled rat serum albumin (FITC-RSA) was injected (intra-arterially) into each rat to permit visualization of the vasculature by fluorescent microscopy. A closed-circuit television system was used to quantitate macromolecular leakage as a change in interstitial fluorescent intensity. Data are given as a relative fluorescent intensity (mean +/- standard error of the mean) in an area of the cremaster with tumor-induced neovascularization. These studies demonstrated that the vasculature induced by rapidly growing Walker 256 carcinosarcoma leak albumin freely when compared with the vasculature induced by the slow-growing chondrosarcoma. Furthermore, there was a significant increase in fluorescent intensity (albumin leakage) in the Walker tumor from 1 minute (24 +/- 3.0) to 30 minutes (49 +/- 5.6). In the normal cremaster area there was a significantly lower fluorescent intensity in the interstitium and a very slight increase with time (4 +/- 1.5 at 1 minute vs. 7 +/- 1.4 at 30 minutes). One interpretation of these data is that the mechanisms responsible for protein leakage from the vasculature of the Walker tumor may be involved in the fast growth and metastases of this tumor as compared with slower-growing tumors such as the chondrosarcoma.     

输卵管癌肉瘤7例分析并文献复习

背景与目的：输卵管癌肉瘤是非常罕见的恶性肿瘤,易转移和复发,预后差。旨在回顾性分析输卵管癌肉瘤的临床表现、组织病理学、治疗和预后,并对既往相关文献进行复习。方法：回顾性分析7例输卵管癌肉瘤患者的临床表现、病理特点和治疗方式,随访生存时间,并复习相关文献。结果：7例输卵管癌肉瘤患者,最常见症状为阴道不规则流血(4/7,57.1%)。病理Ⅰa期1例,Ⅱa期1例,Ⅱb期1例,Ⅲb期l例,Ⅲc期3例。异源性4例(57.1%),均见软骨成分,同源性3例(42.9%)。7例均行瘤体减灭术,6例接受化疗,无放疗病例。存活5例,死亡2例。生存时间超过5年的有3例,最长的至末次随访已存活9年零6个月。结论：病理检查为输卵管癌肉瘤的确诊手段。病理同源性间质成分预后优于异源性间质成分。满意的瘤体减灭术,术后给予足够疗程的TP方案化疗,且全身静脉化疗联合局部腹腔灌注化疗可能是治疗输卵管癌肉瘤的有效治疗方法。     

肺癌肉瘤二例报告并文献复习

目的 :探讨肺癌肉瘤的临床病理特点及其组织来源。方法 :对 2例肺癌肉瘤进行临床病理资料回顾性分析、光镜观察 ,并结合文献对其组织发生、临床表现、诊断及治疗方法进行分析。结果 :肺癌肉瘤主要症状为咳嗽、胸痛、血丝痰等 ,X线示病灶边缘清楚 ,有分叶。镜下见癌与肉瘤共存 ,2例均可见胚胎性支气管结构。结论 :肺癌肉瘤临床特征与肺癌不易区别且预后差 ,外科手术是其主要的治疗手段。组织学特征支持单克隆起源学说。     

Presence of cellular rentinol and retinoic acid binding proteins in experimental rumors.

Cellular retinol and retinoic acid binding proteins were detected in mouse skin papillomas, human adenocarcinoma HAD-1, Dunning Leukemia, Walker 256 carcinosarcoma and mammary adenocarcinoma MAC-1. A chondrosarcoma and Sarcoma 180 apparently contain only the cellular retinoic acid binding protein. Neither protein could be detected in Ehrlich carcinoma and L1210 leukemia. The presence of these proteins might be necessary for sensitivity to retinoid therapy.     

食管梭形细胞癌临床病理分析

目的分析食管梭形细胞癌的临床病理特点,探讨其组织来源。方法回顾性分析4例食管梭形细胞癌的临床病理资料及复习有关文献。结果 4例患者为男性,1例为女性,平均年龄57.2岁,病变位于食管中下段,大体呈息肉型、髓质型、蕈伞型及溃疡型。显微镜下可见鳞状细胞癌和肉瘤样成份,其中3例未找到两者相移行的区域。免疫组织化学检查发现肉瘤样区域细胞中有AE1/AE3、vimentin蛋白表达,息肉型者软骨肉瘤样区域仅有Vimentin和S-100蛋白表达,纤维肉瘤成份仅有vimentin蛋白表达。结论食管梭形细胞癌本质上是1种特殊类型的癌,包括肉瘤样癌和癌肉瘤。肉瘤样癌区域可能是癌细胞双向分化的结果,癌肉瘤的发生机制有待于进一步研究。     

Carcinosarcoma of the ovary: 19 years of prospective data from a single center

BACKGROUND: A review of clinicopathologic features and outcome in women with carcinosarcoma of the ovary (also known as malignant mixed mesodermal tumor [MMMT]) compared with a group of women with serous adenocarcinoma (SAC) of the ovary was conducted. METHODS: Between 1984 and 2002, 1568 patients with epithelial ovarian carcinoma and 70 patients with ovarian carcinosarcoma underwent treatment at the Edinburgh Cancer Centre. Analysis was performed on 65 patients with MMMT, and 746 patients with SAC were selected as a group for comparison. Baseline variables were recorded prospectively and response to chemotherapy and progression-free and cause-specific survival between the groups were compared. RESULTS: Patients with carcinosarcoma had a mean age of 66.6 years, which is significantly older than those with SAC (62.0 years) (P < 0.001). The objective response rate to platinum-based chemotherapy was found to be significantly lower in patients with carcinosarcoma (25% vs. 60%; P = 0.02). Cause-specific survival in the carcinosarcoma group was poor and significantly shorter than that observed in the SAC group (median survival of 8.2 months vs. 20.7 months; P < 0.0001). Progression-free survival in patients with carcinosarcoma also was found to be significantly shorter compared with patients with SAC (median progression-free survival of 6.4 months vs. 12.1 months; P < 0.001). Achieving optimal debulking at the time of initial surgery was found to be a highly significant factor in patients with carcinosarcoma with regard to determining outcome (median survival of 14.8 months for patients with optimally debulked International Federation of Gynecology and Obstetrics Stage III disease vs. 3.1 months for patients with suboptimally/nondebulked Stage III disease; P < 0.001). CONCLUSIONS: Ovarian carcinosarcoma is a distinct entity with a poor prognosis. Patients with carcinosarcoma differ from those with SAC with regard to having an older mean age of onset, an inferior response to platinum-based chemotherapy, and worse progression-free and cause-specific survival. The extent of benefit from chemotherapy is unclear.     

肺癌肉瘤临床特征对生存情况的影响

背景与目的：癌肉瘤（carcinosarcoma, CS）是指一种在同一肿瘤中存有癌和肉瘤两种成分的混合性恶性肿瘤,肺癌肉瘤更是一种罕见的肺部恶性肿瘤,5年生存率低。本研究的目的是探讨肺癌肉瘤的临床特征对生存情况的影响。方法：通过收集64例肺癌肉瘤患者的临床资料,回顾性分析了患者的性别、年龄、p-TNM分期、肿瘤位置、化疗和手术方式等因素对预后的影响。结果：64例肺癌肉瘤患者术后5年生存率为14.1%。早期（Ⅰ、Ⅱ）和晚期（Ⅲ、Ⅳ）组5年生存率分别为28.6%和2.8%,差异有统计学意义（P=0.003）;全肺切除、肺叶切除及姑息性手术组的5年生存率分别为33.3%、9.5%和0,差异有统计学意义（P=0.039）。结论：p-TNM分期、手术方式与肺癌肉瘤的生存情况关系密切。早期发现,早期行根治性手术,对延长患者的生存非常有意义。     

Mixed mesodermal tumor of the ovary

A 68-year-old woman with a malignant mixed mesodermal tumor of the ovary is reported. Her clinical course worsened rapidly and she died 3 months after admission. At autopsy, the pelvic cavity was seen to be filled with a huge tumor mass. Histologically, the tumor contained malignant epithelial and mesenchymal elements. The epithelial component was an adenosquamous carcinoma. The stromal element was comprised mainly of anaplastic, undifferentiated spindle cells. A variety of malignant mesenchymal elements were also present. They resembled rhabdomyosarcoma, liposarcoma, chondrosarcoma, fibrosarcoma, and myxosarcoma . A review of the Japanese literature concerning this tumor is also presented.     

20例食管癌肉瘤的诊断与治疗分析

目的食管癌肉瘤是一种罕见病,通过回顾分析本组较大样本食管癌肉瘤的病理及临床特点,探讨其诊断治疗.方法对20例食管癌肉瘤病人的临床资料进行回顾性分析.结果食管癌肉瘤多为覃伞样、息肉样腔内生长,个别呈浸润生长.光镜下肉瘤与癌两种成份共存,瘤体以肉瘤成分为主,表面及蒂多分布癌组织,活检常显示为鳞癌,故术前多不能明确诊断.20例患者中9例侵犯粘膜层,1例浅肌层,4例深肌层,6例侵犯外膜.4例出现淋巴结转移,淋巴转移率20%,转移成分3例鳞癌,1例肉瘤.手术切除率100%.1、3、5年生存期分别为85%、68%、68%.结论食管癌肉瘤是一种低侵袭性,低淋巴转移率,预后尚佳的肿瘤.X线表现为食管腔内息肉样的充盈缺损,轮廓较为光滑整齐,粘膜显示"涂抹征".食管镜活检多显示鳞癌或低分化癌,术前难以确诊.治疗以手术切除为主,必要时辅以放化疗.     

1513例骨肿瘤及肿瘤样病变统计分析

 本文统计分析了骨肿瘤及肿瘤样病变1513例。其中良性骨肿瘤907例,占59.95%;恶性骨肿瘤450例,占29.74%;肿瘤样病变156例,占10.31%。三组病例都好发于11—40岁,男性较女性多见,为2。28:1。好发部位较多见于股骨、胫骨和肱骨,不同病变有不同的好发部位。良性骨肿瘤中以骨软骨瘤最多见,其次为骨巨细胞瘤。恶性骨肿瘤中以骨肉瘤最多见,其余依次为软骨肉瘤、转移癌、尤文氏瘤、骨髓瘤、骨纤维肉瘤、巨细胞瘤、脊索瘤、皮质旁骨肉瘤、网织细胞肉瘤。骨的肿瘤样病变包括嗜伊红性肉芽肿、骨纤维异常增殖症,弧立性骨囊肿和动脉瘤样骨囊肿。     

Differential expression of matrix metalloprotease-7 in each component of uterine carcinosarcoma

The aim of this study was to investigate the expression of matrix metalloprotease-7 (MMP-7) in each component of uterine carcinosarcoma. Surgical specimens of primary tumors of uterine carcinosarcomas were obtained from 13 patients. The carcinomatous component consisted of adenocarcinoma with or without squamous differentiation. The sarcomatous component consisted of spindle cell sarcoma, chondrosarcoma and rhabdomyosarcoma, either alone or in combination. The immunohistochemical expression of MMP-7 protein was examined using the avidin-biotin peroxidase complex technique employing the anti-MMP-7 monoclonal antibody. Expression of MMP-7 protein was detected in 9 (69.2%) of 13 adenocarcinoma components, while no staining was observed in any of the sarcomatous components examined. There was a statistically significant difference of the positive rate for MMP-7 between epithelial components and sarcomatous components of uterine carcinosarcoma (p<0.01). In some cases, MMP-7 was abundantly expressed at the invasive front of adenocarcinomas. It is concluded that MMP-7 protein is differentially expressed between the carcinomatous component and the sarcomatous component of uterine carcinosarcoma. Each component of carcinosarcoma may have its own potential for invasion and metastasis. MMP-7 may contribute to the invasive nature or growth capacity of the carcinomatous component of uterine carcinosarcoma, while it may not have a relation to that of the sarcomatous components.     

Clinico pathologic analysis of 1503 thyroid tumors]

A clinicopathologic analysis of 1503 thyroid tumors was conducted revealing 82.9% of benign and 17.8% of malignant lesions. The most common type of benign tumor was simple adenoma (62.1%) and of malignant tumor, papillary adenocarcinoma (52.5%). The malignant to benign tumor ratio was 1:4.8. The male to female sex ratio was 1:2.6 in the malignant and 1:3.8 in the benign tumors. The overall 5- and 10-year survival rates for all cancers were 67.7% and 47.6%, respectively. It is suggested that the follicular carcinoma could further be divided into well differentiated (papillary and follicular), poorly differentiated (including squamous cell carcinoma) and undifferentiated types.     

Squamotous-type sarcomatoid carcinoma of the lung with rhabdomyosarcomatous components

Lung carcinosarcoma is an infrequently biphasic tumor composed of carcinomatous and sarcomatous components. It is divided into endobronchial (squamous-type) and peripheral (glandular type) categories. The carcinomatous component is usually a squamous carcinoma, and the sarcomatous component usually resembles a fibrosarcoma or a malignant fibrous histiocytoma. The presence of rhabdomyoblastic differentiation in such neoplasms is exceedingly rare. There are strong associations with smoking and asbestosis. In this study, we describe a unique case of a 43-year-old man with a 75 packet/year smoking history in whom a rare mixed malignant tumor of the lung was diagnosed and treated by left pneumonectomy. Histological examination of the resected specimen showed squamous cell carcinoma and rhabdomyosarcoma components. Although rare, the association of a sarcomatoid carcinoma of the lung with squamous cell carcinoma and rhabdomyosarcomatous component is possible and should be kept in mind when dealing with these unusual tumors.     

CYFRA 21-1 serum analysis in patients with esophageal cancer.

This study was conducted to determine a potential use of CYFRA 21-1 in patients suffering from carcinoma of the esophagus. CYFRA 21-1 serum concentrations of 50 patients with histologically proven malignant lesion of the esophagus were compared with 50 healthy persons, 50 patients with benign esophageal disease, and 50 patients with benign lung disease. Additional analysis of serum carcinoembryonic antigen, CA 72-4, and squamous cell carcinoma-antigen serum concentrations were performed. The patients with esophageal carcinoma underwent follow-up tumor marker examinations every three months for 1 year. Analysis to detect statistically significant differences was conducted to estimate a cutoff and to evaluate tumor entity, tumor stage, survival, and tumor-free survival. CYFRA 21-1 at a cutoff of 1.40 ng/ml showed an overall sensitivity to esophageal carcinoma of 36% (45.5% to squamous cell carcinoma, 17.6% to adenocarcinoma) at a specificity of 97.3%. CYFRA 21-1 concentrations showed a tendency to higher serum levels depending on local tumor burden. A correlation of CYFRA 21-1 with various N- or M-stage disease was not observed. Postoperative development in terms of survival and tumor-free survival showed significant correlation to preoperative CYFRA 21-1 concentrations. Clinical tumor recurrence was preceded by CY-FRA 21-1 elevation by 3.4 months. For prognosis and follow-up, this marker is justified for additional analysis in a larger series of patients suffering from carcinoma of the esophagus.