心脏原发性血管肉瘤3例临床病理学观察

目的探讨心脏原发性血管肉瘤的临床病理学特征及鉴别诊断,提高对该肿瘤的认识。方法回顾性分析3例发生在心脏的血管肉瘤的临床资料、病理形态学、免疫组化结果,并复习相关文献。结果 2例患者为男性,1例患者为女性,年龄33~51岁;均发生在右心房,1例发生肺转移。临床表现无特异性,表现为胸闷、心慌、气促和呼吸困难。镜下瘤组织主要由梭形、卵圆形细胞构成,呈团块、条索状、乳头状结构排列或围成不规则、互相吻合的血管腔,出血及坏死多见。免疫组化显示肿瘤细胞:CD31(+),CD34(+),FⅧ(+)。结论心脏原发性血管肉瘤非常罕见,由于其临床表现无特异性,早期诊断困难,确诊主要依赖于病理组织学及免疫组化检查。     

肺原发性癌肉瘤21例临床病理分析

原发性肺癌肉瘤21例。本病好发于中老年男性,肺上叶较易累及。中央型略多于周围型,周围型一般肿瘤较大。临床症状出现较晚,明确诊断时往往已有转移。最早出现的转移部位为肺门淋巴结,胸膜及纵隔亦可累及。手术切除后预后较差。 恶性上皮成分通常为鳞癌,其次为腺癌,小细胞未分化癌。间质成分以纤维肉瘤最多风。     

肺癌肉瘤的临床病理研究

本文报道了临床罕见的肺癌肉瘤的病理检查结果，并进行了探讨研究。     

心脏原发性血管肉瘤9例临床病理分析

目的:探讨心脏原发性血管肉瘤的临床病理特点、诊断和鉴别诊断、治疗及预后特点。方法:对9例心脏原发性血管肉瘤进行光镜形态及免疫组化特征观察,并结合文献分析讨论。结果:9例中,男性5例,女性4例,年龄在19~62岁,平均39岁。镜下见肿瘤形态多样,局部呈大小不规则的血窦样结构,互相连通,腔内壁衬覆肿瘤性内皮细胞,细胞异型性明显,免疫组化:CD31(+),CD34(+),Fli-1(+),ERG(+),Vimentin(+)。结论:心脏原发性血管肉瘤是一种罕见的预后很差的恶性肿瘤,临床表现缺乏特异性,病理形态多样,诊断困难,借助免疫组化特异性标记有助于明确诊断。     

肺血管肉瘤的诊断与治疗——附1例报告并文献复习

目的复习肺血管肉瘤的临床表现、病理形态学特点、诊断、鉴别诊断和治疗。方法结合文献报道,回顾性分析5例肺血肉瘤患者临床资料。结果肺血管肉瘤临床症状为胸痛、咳嗽、咯血,肺内多发结节、斑片影,病理检查瘤体主要为血管内衬异型的瘤细胞（血管内皮细胞）,可见相互吻合大小不一形态不规则的血管网,免疫组化CD34、CD31和vimentin阳性。结论肺血管肉瘤是罕见的软组织高度恶性肿瘤,预后差。肺血管肉瘤需与肺腺癌、肺结核、真菌等疾病鉴别。     

肺血管肉瘤的诊断与治疗——附1例报告并文献复习

目的复习肺血管肉瘤的临床表现、病理形态学特点、诊断、鉴别诊断和治疗。方法结合文献报道,回顾性分析5例肺血肉瘤患者临床资料。结果肺血管肉瘤临床症状为胸痛、咳嗽、咯血,肺内多发结节、斑片影,病理检查瘤体主要为血管内衬异型的瘤细胞(血管内皮细胞),可见相互吻合大小不一形态不规则的血管网,免疫组化CD34、CD31和vimentin阳性。结论肺血管肉瘤是罕见的软组织高度恶性肿瘤,预后差。肺血管肉瘤需与肺腺癌、肺结核、真菌等疾病鉴别。     

肺肉瘤样癌35例病理特点分析

目的明确肺肉瘤样癌的病理特点。方法对35例经病理学证实的肺肉瘤样癌患者的病理及免疫组化特点进行回顾性分析。结果本组患者肺肉瘤样癌发生于左肺11例,右肺23例。病理亚型以多形性癌(27例)为主,即鳞状细胞癌、腺癌伴有梭形细胞和(或)巨细胞成分。免疫组化检查显示上皮细胞角蛋白(CK)阳性28例、波形蛋白阳性26例。结论肺肉瘤样癌病理类型以多形性癌为主。     

胸膜血管肉瘤的诊治

血管肉瘤(angiosarcoma)也称恶性血管内皮瘤,是由血管内皮细胞或向血管内皮细胞方向分化的间叶细胞发生的恶性软组织肿瘤,占所有软组织肉瘤的1%~2%[1]。血管肉瘤可累及全身多个器官,通常发生在皮肤、深层软组织[2],也可累及头颈部、乳房、脾脏和肝脏、肺部和胸膜等部位[3-5]。原发性胸膜血管肉瘤非常罕见,目前相关文献病例数报道较少,且部分患者资料不全。原发性胸膜血管肉瘤的主要症状为呼吸困难、胸闷及胸痛[6],但其临床表现缺乏特异性,容易导致漏诊、误诊。本文就胸膜血管肉瘤的特点及目前对该疾病的认识进行分析和总结。     

肺转移性脂肪肉瘤1例及文献复习

目的 探讨肺转移性脂肪肉瘤的临床和病理特点,减少误诊、漏诊.方法 对收治的1例肺转移性脂肪肉瘤患者的临床资料进行回顾性分析.结果 本例为55岁男性,因“咳嗽伴气促1个月,加重1d”入院.CT和胸水B超示右侧包裹性胸腔积液,肿瘤声学造影示右肺占位.胸外科手术切除右肺肿块,术后病理提示圆细胞型脂肪肉瘤.术后患者出现四肢、臀部、腹部巨大肿块,且生长迅速,术后给予放化疗及对症支持治疗.结论 肺原发性和转移性脂肪肉瘤临床均少见,症状隐匿、缺乏特异性,易误诊,B超肿瘤声学造影有助于早期鉴别.除手术切除外,生长迅速、多发转移者可考虑术前、术后联合放化疗.     

Clinical manifestations of primary hepatic angiosarcoma

Malignant tumors of the liver stemming from mesenchymal origins are rare neoplasms, <1% of primary malignant hepatic lesions. Primary hepatic angiosarcoma (PHA) is the most common (36%). This study describes the incidence and clinical characteristics of this rare tumor in two medical centers, over the past 18 years. We reviewed tumor registry files at Jackson Memorial Hospital and oncology data records at Cedar's Medical Center, 1979–1997. A total of 865 primary hepatic tumors were identified, of which five cases (0.58%) were PHA; four were men, and the median age was 53 years. Symptoms and signs included: pain, anemia, fever of unknown origin, weight loss, abdominal mass, and hemoperitoneum. Median survival was only 6 months. In conclusion, primary hepatic angiosarcomas frequently are symptomatic. The presentation and preexisting factors are valuable in establishing a clinical suspicion to diagnose this rare tumor. Although imaging studies are helpful, they are not conclusive, and liver biopsy is usually required.     

Characteristics and outcomes of primary pleural angiosarcoma: A retrospective study of 43 published cases

Primary pleural angiosarcoma (PPA) is an extremely rare malignancy for which there is no consensus on treatment. The clinical course of PPA is usually quickly fatal, regardless of the treatment used.We summarized and evaluated a relatively large population of published PPA cases to assess prognostic factors, diagnostic approaches, treatment methods and clinical outcomes. Using the CNKI, Embase, and PubMed databases, literature published in English and Chinese from 1988 through 2020 was searched using the terms “primary pleural angiosarcoma,” “pleural angiosarcoma,” and “pleuropulmonary angiosarcoma.”A total of 43 patients with PPA were identified in retrospective case series and case reports. The median age at diagnosis was 64 years (range 24–87 years), and the median overall survival was 4 months (range 0.1–180 months). Approximately 80% of patients died from PPA within 10 months of diagnosis, and the 2-year survival rate was approximately 4.4%. In univariate analyses, the presence of pleural effusion and hemothorax were significant predictors of decreased survival, with hazard ratios (HRs) of 2.7 (P = .04) and 3.3 (P = .006), respectively. Sixteen patients received no therapy, and their prognosis was worse than patients who did receive therapy (P = .019). Radiation therapy improved survival more than no radiation therapy (P = .007). Patients appeared to derive clinical benefit from chemotherapy (P = .048). However, tumor resection did not seem to provide a survival benefit (P = .051). In multivariate analysis, tumor resection, and radiation were independent, statistically significant, positive predictors of better survival, with HRs of 0.3 (P = .017) and 0.1 (P = .006), respectively. The presence of hemothorax was an independent predictor of worse prognosis (P = .006).Primary angiosarcoma of the pleura is a rare, poorly understood malignancy with a poor prognosis; hence, the clinical spectrum of PPA is not completely defined. By multivariate analysis, this retrospective study showed a survival benefit of tumor resection or radiation therapy, and the presence of hemothorax was a significant prognostic factor for poor outcomes.     

Cardiac angiosarcoma arising in a coronary artery: Angiographic and pathologic findings

We discuss a unique case of an angiosarcoma which arose in an atherosclerotic coronary artery. It was widely metastatic. We describe the angiographic findings and their differential diagnosis, and briefly discuss this relatively common cardiac malignancy.     

Five years and 4 months of recurrence-free survival in hepatic angiosarcoma

A 54-year-old woman was referred with the diagnosis of hepatic angiosarcoma, made by percutaneous biopsy under ultrasonographic guidance. Ultrasonography (US) had revealed a 48 × 42 × 35?mm mass in the right lobe. Standard biochemical tests and whole blood count had yielded normal results. At our institution, magnetic resonance imaging demonstrated a hypervascular mass in the right lobe. Alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 levels were normal. Serological tests were negative for hepatitis B and C viruses. There was no evidence of metastasis. A right hepatectomy was performed. Histopathological examination confirmed the diagnosis of angiosarcoma. However, there was a suspicion of microscopically positive margins. Relaparotomy and resection of a 1-cm-thick slice of hepatic parenchyma was performed. Histopathological examination revealed necrotic tumor cells at the previous margin. The new surgical margin was tumor free. Due to the expected poor prognosis, prophylactic chemoembolization of the remnant liver (lipiodol + adriamycin + mitomycin) was performed at 3 and 7 months postoperatively. She has been alive without recurrence for 5 years and 4 months. Hepatic angiosarcoma has two distinct presentations: multiple tumors and a solitary tumor. The reported poor results largely stem from the predominance of the multiple tumors and consequent unresectability. Long-term survival is possible in solitary resectable hepatic angiosarcomas.     

Case report of primary splenic angiosarcoma with hepatic metastases

Primary splenic angiosarcoma(PSA) is the most unusual type of malignancy with early multifocal metastasis through hematogenous spread. PSA is generally believed to originate from splenic sinusoidal vascular endothelium with a high rate of metastasis and to have a poor prognosis. Its etiology and pathogenetic mechanisms have not yet been clearly described. Thus far, only approximately 200 cases have been reported. PSA has variable symptomatology with the potential to present with life-threatening complications. The diagnosis of PSA is challenging; and often late. PSA should be considered in the differential diagnosis of patients with splenomegaly and anemia of unknown etiology. Surgical treatment with splenectomy is considered the only curative intervention for potential long-term disease-free survival. Early diagnosis and treatment are very important. It is important that clinical doctors improve the understanding of PSA. Herein, we report one rare case of PSA with hepatic metastases, along with a review of the current literature.     

Spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis

Aim: We report herein a case of spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis and review the correlative literature. Methods: The resected specimen was examined by histopathological and immunohistochemical evaluation. Results: The final diagnosis was spontaneous ruptured primary hepatic angiosarcoma coincident with Schistosoma Japonica liver fibrosis Conclusion: Considering the nature of primary hepatic angiosarcoma, in particular the ruptured hepatic angiosarcoma, it is obviously desirable to avoid any unnecessary delay or definitive surgical treatment. It is presumed that angiosarcoma in the liver has a possible association with S. japonicum and the deposition of ovae in liver.     

Contrast uptake in primary hepatic angiosarcoma on gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging in the hepatobiliary phase

Primary hepatic angiosarcoma is the most common malignant mesenchymal tumor of the liver. It has a poor prognosis and various appearances on magnetic resonance(MR) images. We report a case of hepatic angiosarcoma with a characteristic appearance on gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid(Gd-EOB-DTPA)-enhanced MR imaging in the hepatobiliary phase. A 72-year-old man was admitted with a complaint of abdominal pain. Gd-EOBDTPA-enhanced MR imaging revealed a liver tumor that showed slight hyperintensity in the hepatobiliary phase. These findings suggested Gd-EOB-DTPA uptake in the tumor. An autopsy revealed the solid proliferation and sinusoidal spreading of hepatic angiosarcoma cells. Immunohistochemistry indicated that the tumor was negative for OATP1B3. Gd-EOB-DTPA uptake in the liver tumor in the hepatobiliary phase suggested sinusoidal tumor invasion with residual normal hepatocytes.