Angiosarcoma of the scalp: Report of two cases with fatal pulmonary complications and a review of Japanese autopsy registry data

Summary Two cases of angiosarcoma of the scalp were reported. The patients were elderly men and died from pulmonary complications, including pneumothorax, pulmonary haemorrhage and pneumonia, associated with metastatic tumours in the lungs. The data recorded from 95 autopsies of patients with angiosarcoma in Japan during 1980–1984 were analyzed. According to the anatomical distribution of the primary tumour, the patients could be subdivided into a scalp group and non-scalp group. In both groups, the most common metastatic site was the lung. The patients of the scalp group had more frequent pulmonary complications such as pneumonia, haemothorax, atelectasis and pneumothorax, when compared with the patients of the non-scalp group. In particular, pneumothorax was observed only in the patients of angiosarcoma of the scalp. The results indicate that angiosarcoma of the scalp tends to metastasize to the lung, especially to the subpleural or surface pleural area, and these metastatic tumours are prone to necrosis, causing characteristic pulmonary complications.Supported in part by a grant-in-aid for Cancer Research from the Ministry of Education, Science and Culture of Japan     

Epithelioid angiosarcoma of the intestinal tract with endothelin-1-like immunoreactivity

Two rare cases of intestinal epithelioid angiosarcoma arising in the sigmoid colon and small intestine are reported. The small intestinal tumours were located in the duodenum and upper jejunum. All tumours showed reddish-black protuberant masses with comparatively clear margins. Histology reveals solid and epithelioid growth of large polygonal cells, mimicking undifferentiated carcinoma. Vascular differentiation such as lumina containing red cells and intracytoplasmic vacuolization is noticed. Intense immunoreactivity toUlex europaeus agglutinin I and JC70 (CD31), and sporadic positive reaction to factor VIII related antigen were detected in one case but not in the other. However, both tumours have cells which show intense endothelin-1 immunoreactivity. Positive immunostaining occurred with low molecular weight keratins suggesting epithelial differentiation and suggested epithelioid angiosarcoma as a diagnosis, a specific entity within angiosarcoma. Because of their histological features, epithelioid angiosarcomas may be confused with undifferentiated carcinomas. Endothelin-1-like immunoreactivity seems to be a good marker for this type of angiosarcoma, even when the tumour cells lack usual marker substances for endothelial cells.     

Sarcoma de Kaposi y angiosarcoma cutáneo: directrices para el diagnóstico y tratamiento

Kaposi sarcoma is a vascular sarcoma with 4 clinical variants: classic Kaposi sarcoma, which mainly affect the extremities of elderly patients and follows a chronic, generally indolent course; African Kaposi sarcoma; immunosuppression-associated Kaposi sarcoma; and AIDS-associated Kaposi sarcoma. Type 8 human herpesvirus is the etiologic agent in all 4 variants. Cutaneous angiosarcoma is a cutaneous neoplasm with a very poor prognosis. It carries a high probability of local relapse and has a 10% to 15% survival rate at 5 years. There are 3 main variants of cutaneous angiosarcoma: idiopathic angiosarcoma of the face and scalp; Stewart-Treves syndrome; and postradiation angiosarcoma. The only potentially curative treatment is surgery with or without radiotherapy. However, its indistinct borders and multicentric nature mean that treatment is often palliative with chemotherapy, radiotherapy, or both.     

血管肉瘤的分子靶向及免疫治疗研究进展

血管肉瘤(AS)是一种起源于血管或者淋巴管内皮细胞的少见的分化差的软组织肉瘤,预后不佳.目前医学界对其治疗方案未达成共识,其主要治疗手段仍以手术、放化疗为主,但患者获益不佳.随着分子生物学的飞速发展,新的分子靶点被逐渐发现,部分患者可从中获益.另外,随着肿瘤免疫治疗的开展,AS的治疗手段得到极大丰富.本文综述了血管肉瘤...     

Primary splenic angiosarcoma with liver metastasis: A case report and literature review

Primary splenic angiosarcoma(PSA) is an unusual and highly malignant vascular tumour with a high rate of metastatic. Moreover, the research on prognosis of the disease is poor. The epidemiology, etiology, clinical diagnosis and treatment of the disease remain challenging, because case reports of the disease are few in number. In accordance with other malignant tumors, PSA is very aggressive, and the majority of patients in which this disease is found are at an advanced stage. Almost all patients die within 12 mo of diagnosis irrespective of treatment. We report here a woman who had complained of upper bellyache and anorexia for 10 d. Magnetic resonance imaging showed enlargement of the spleen with multiple heterogeneous masses in the lower pole of the spleen. A hand-assisted laparoscopic splenectomy was performed which allowed histopathologic diagnosis. The patient was diagnosed with PSA and liver metastasis, and succumbed to the disease 35 d after surgery. The literature was finished combined with the clinical features, diagnosis and management of PSA.     

Angiosarcoma of the tongue: A case series and literature review

Purpose

Angiosarcoma of the tongue is an exceedingly rare malignancy of the head and neck. Such lesions can be primary in nature or occur in a previously irradiated field. We examine a series of cases with relation to clinical presentation, diagnosis, management, and outcomes.

头皮血管肉瘤16例临床病理及治疗预后分析

目的：探讨头皮血管肉瘤临床病理学特征,并分析肿瘤分期、治疗与预后的相关性。方法分析2002年9月至2013年6月东南大学附属中大医院整形烧伤科收治的16例原发于头皮且无转移的血管肉瘤患者的临床及随访资料。按照 AJCC2010年第7版软组织肉瘤分期标准分期,Kaplan-Meier 法计算生存率,Log-Rank 法绘制生存曲线,Cox 模型进行多因素回归分析。分析患者生存率以及预后的影响因素。结果16例患者中,GTNM分期Ⅰ期4例,Ⅱ期4例,Ⅲ期8例。临床早期常在头面部出现淤斑样皮损,后期发生浸润性暗红斑,伴结节及溃疡,易破溃出血。组织病理检查示真皮广泛血管增生及浸润,组织形态变异较大,常见细胞异形。出现复发的中位时间为15个月,7例局部复发,8例远处转移,转移的中位时间为20.5个月,转移至肺4例,淋巴结2例,肝1例,骨1例。生存时间 I 期4例为（33.0±4.4）个月（中位数32个月）,Ⅱ期4例为（24.0±7.9）个月（中位数15个月）,Ⅲ期8例为（23.9±3.9）个月（中位数24个月）。单纯手术治疗5例生存时间为（23.4±5.2）个月（中位数21个月）,手术+放射治疗6例为（24.4±5.7）个月（中位数24个月）,手术+放射治疗+免疫治疗4例为（35.8±9.7）个月（中位数26个月）。结论头皮血管肉瘤的治疗以手术基础上辅以放射+免疫治疗为首选。影响原发性头皮血管肉瘤预后的主要因素为肿瘤大小和治疗方式。     

Anti-tumor effect of PDT using Photofrin in a mouse angiosarcoma model

Angiosarcoma, a malignant tumor of vascular endothelial cell origin, is a lethal disease for which complete cure is rarely seen. The objective of this study was to determine the efficiency of photodynamic therapy (PDT) as a new treatment for angiosarcoma. PDT (630 nm, 25 J/cm²) using Photofrin for a mouse angiosarcoma cell line of human origin (ISOS-1) showed that the rate of cell death increased with increase in the concentration of a photosensitizer (LD₅₀: ∼2 μg/ml). Furthermore, PDT (630 nm, 100 J/cm²) with Photofrin (5 mg/kg, i.v.) in mice transplanted with ISOS-1 cells resulted in complete disappearance of the tumor in 40% of mice and marked inhibition of tumor growth in the remaining 60%. Significant increases in TUNEL-positive cells and Ki-67-positive cells ware seen 4 h after PDT, indicating that PDT led to not only cell death but also inhibition of the proliferation of angiosarcoma cells. The results show that PDT is effective for treatment of angiosarcoma.     

脾脏原发性血管肉瘤1例及文献复习

脾脏原发性血管肉瘤罕见且预后极差,临床虽强调早确诊、早治疗,但其临床表现缺乏特异性,易于误诊,现阶段临床确诊主要依靠手术全切脾脏后组织病理学检测。为提升诊断效果,临床应探讨脾脏原发性血管肉瘤的一般表现并找到可靠的诊断方案。本研究以1例手术病理诊断确诊患者为研究对象,重点探讨免疫组化技术在该疾病诊断中的价值。该病例行影像学检测可见脾脏内多发性实性占位,CT于增强后明显强化,手术标本肉眼观察（巨检）及镜检均有一定特征,行免疫组化反应可见FⅧRag（+）、CD34（+++）、CD31（+++）、Vim（+++）、SMA（局灶+）,其免疫组化特征可与上皮样血管内皮细胞瘤、脾窦岸细胞血管瘤等疾病有效区分,有助于指导临床诊断。