混合性血管内皮瘤1 例报告及文献复习

 目的 探讨混合性血管内皮瘤临床病理特点及免疫组化表型。方法 对1例混合性血管内皮瘤病理组织学及免疫组化表型中的CD31、CD34、VⅢ因子、VWF、PCK、Vimentin、EMA进行观察并复习相关文献。结果 巨检：送检骨骼肌带筋膜，肌间见31个灰白色结节，直径0．6～2．5cm，切面灰白色鱼肉状。镜检：瘤组织成分复杂，有网状型血管内皮瘤、上皮性血管内皮瘤及梭形细胞血管内皮瘤成分。免疫组化表型为血管来源肿瘤。结论 混合性血管内皮瘤属极其罕见低度恶性肿瘤，成分多样。免疫组化表型有助于其诊断分析。     

Hepatic haemangioendothelioma in adults: excellent outcome following liver transplantation

Hepatic epithelioid haemangioendotheliomas (HEHEs) are rare, low-grade vascular tumours. Five adults with HEHEs and one adult with a vascular tumour showing combined features of haemangioma and haemangioendothelioma underwent liver transplantation. Two HEHE patients had extrahepatic metastases at the time of transplantation. Median survival time following diagnosis was 10.7 years (range 40 months to 195 months). One patient needed resection of a HEHE in the breast 13 years post-transplantation. All six patients are surviving free from disease 22 to 166 months after transplantation (median 77 months). One HEHE-patient who had been treated for 8 years for vertebral and cerebral localisations is free of disease without immunosuppression 56 months after transplantation. We can conclude that liver transplantation is a valuable treatment for hepatic haemangioendothelioma, even in cases of extrahepatic localisation of the disease.Giuseppe Orlando is a transplant research fellow from the University of Rome, Tor Vergata (It)     

Vaginal haemangioendothelioma: an unusual tumour

Vaginal tumours are uncommon and this is a particularly rare case of a vaginal haemangioendothelioma in a 38-year-old woman. Initial presentation consisted of symptoms similar to uterovaginal prolapse with “something coming down”. Examination under anaesthesia demonstrated a necrotic anterior vaginal wall tumour. Histology of the lesion revealed a haemangioendothelioma which had some features of haemangiopericytoma. While the natural history of vaginal haemangioendothelioma is uncertain, as a group, they have a propensity for local recurrence. To our knowledge this is the third reported case of a vaginal haemangioendothelioma. Management of this tumour is challenging given the paucity of literature on this tumour. There is a need to add rare tumours to our “knowledge bank” to guide management of these unusual tumours. The patient has given written informed consent to the publication of the article.     

Neonatal hepatic haemangioendothelioma: treatment options and dilemmas

We describe a case of rapidly progressive neonatal diffuse hepatic haemangioendotheliomas. The clinical picture was characterized by respiratory insufficiency due to gross abdominal enlargement, coagulopathy, and the development of cardiovascular insufficiency during the course of disease. Pharmacological, radiotherapeutic and endovascular treatment options all proved ineffective. We describe our own experience and the steps taken to treat this child. Unfortunately, the child died as a consequence of the disease. As cases like this are rare and are associated with severe morbidity and high mortality, more knowledge needs to be gained on infantile hepatic haemangioendotheliomas and their optimal treatment. The use of a web-based data registry could be beneficial.     

Epithelioid haemangioendothelioma of the brain: a case report

We report a rare epithelioid haemangioendothelioma in the frontal lobe. The CT and MRI findings are characteristic and correspond to histological features intermediate between those a cavernous haemangioma and an aggressive vascular tumour. Received: 20 November 2000/Accepted: 10 January 2001     

Primary cardiac hemangioendothelioma of the mitral valve

Primary cardiac hemangioendothelioma is a very rare tumor. Up to now, <20 cases have been reported worldwide; involvement of mitral valve by the tumor is extremely rare. In this report, a case of hemangioendothelioma arose from the mitral valve and was successfully resected, and after few months the tumor recurred and infiltrated the heart.     

Angiosarcoma of skull base in a 1-year-old child—A case report

Angiosarcoma is a rare tumour of endothelial cell origin whilst malignant skull base tumours are highly unusual in paediatric patients. This case reports an angiosarcoma involving the clivus and basi-sphenoid region of the skull base, in a 1-year-old boy. This tumour is extremely rare in childhood, particularly in this site. The histological features were consistent with a high-grade haemangioendothelioma, categorised as an angiosarcoma [1]. The characteristics of this rare malignancy and the challenges in its management are discussed. This is the youngest reported patient, to our knowledge, with an angiosarcoma of the skull base.     

Hepatic haemangioendothelioma presenting with early heart failure in a newborn: treatment with hepatic artery embolization and interferon

We report our management of a newborn presenting in utero with congestive heart failure secondary to a giant hepatic haemangioendothelioma. Control of heart failure was achieved by transcalheter hepatic artery embolization, and rapid regression of the tumour was observed after a course of α₁ interferon. The combination of hepatic artery embolization and interferon may be a useful approach in the management of this rare, potentially fatal condition.