Epithelioid sarcoma‐like (pseudomyogenic) hemangioendothelioma,clinically mimicking dermatofibroma,diagnosed by skin biopsy in a 30‐year‐old man

Epithelioid sarcoma‐like (pseudomyogenic) hemangioendothelioma (ESHE) represents a rare soft tissue and bone tumor that typically presents as nodule(s) in the distal extremities of young adults. The nodules traverse several tissue planes simultaneously and can involve the dermis, subcutis, skeletal muscle and bone. ESHE shares clinical and microscopic features with epithelioid sarcoma (ES), and, accordingly, is commonly misdiagnosed as ES. However, unlike ES, which has a poor prognosis, ESHE commonly follows an indolent course. Herein, we report a case of ESHE diagnosed by skin biopsy that clinically mimicked a dermatofibroma. We also provide clinical photographs of the lesions in various stages of development, representing information that has not been previously published, to our knowledge.     

Update on cutaneous epithelioid vascular tumours

Cutaneous epitheiloid vascular tumours are heterogeneous groups of vascular proliferations sharing in common the epithelioid morphology of tumour cells. Based on the WHO classification epithelioid vascular tumours are classified on the basis of their biological behavior into benign tumours (epithelioid haemangioma, epithelioid angiomatous nodule) and malignant tumours (epithelioid haemangioendothelioma and epithelioid angiosarcoma). While cutaneous epithelioid haemangioendothelioma affecting only the skin usually but not always follows an indolent clinical course, cutaneous epithelioid angiosarcoma is generally a highly aggressive tumour with dismal prognosis. Also included in this review is pseudomyogenic haemangioendothelioma, a vascular tumour of intermediate (rarely metastasizing) malignancy. It is not traditionally grouped under vascular tumours with epithelioid morphology. Nevertheless, pseudomyogenic haemangioendothelioma is discussed herein because it often contains a proportion of cells with epithelioid morphology, mimicking other epithelioid tumours of different lineage. Correct recognition of cutaneous epithelioid vascular tumours can be difficult due to their overlapping histological features and often absent or limited formation of open vascular spaces, but is crucial for correct management of the patients. In this paper, we review the main clinicopathological and immunohistochemical features of cutaneous epithelioid vascular tumours, including pseudomyogenic haemangioendothelioma, discuss their crucial differential diagnosis, analyze treatment options and prognosis, and give an update on molecular genetic features of this distinctive group of vascular proliferations.     

多手指假肌源性血管内皮瘤一例

患者男,21岁,因右手多指发生皮下肿物伴疼痛5个月就诊。肿物初起为一针头大红斑,仅累及小指,无疼痛等不适。发病3个月后红斑迅速长大,呈结节状,数量爆发性增多,并累及无名指、拇指。皮损部分聚集,呈白色或黄色,遇冷及接触硬物后呈电击样或针刺样疼痛。患者发病以来未见其他不适,既往史及家族史无特殊。体检：一般情况可,浅表淋巴结未触及,心、肺、腹检查未见异常。皮肤科检查：右手小指见10个直径0.2 ~ 0.8 cm黄色或白色结节,部分皮损聚集,但不融合……     

Rarely metastasizing soft tissue tumours

Soft tissue tumours that rarely metastasize have been afforded their own subcategory in recent WHO classifications. This review discusses the nature of these tumours and the difficulty in constructing usefully simple classifications for heterogeneous and complex groups of tumours. We also highlight the specific rarely metastasizing soft tissue tumours that have been recently added to the WHO classification (phosphaturic mesenchymal tumour, pseudomyogenic haemangioendothelioma) and those entities where there have been recent important defining genetic discoveries (myxoinflammatory fibroblastic sarcoma, solitary fibrous tumour, myoepitheliomas).     

Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma

Pseudomyogenic hemangioendothelioma (PMH) is a rare, mostly indolent vascular tumor. Extensive cases are treated with amputation as chemotherapy seems to be ineffective. Recently, promising results were published using mammalian target of rapamycin (mTOR) inhibitors in tumors of vascular origin. Here, we present a case of a child with advanced PMH relapsing after surgery and chemotherapy. Sirolimus achieved significant clinical improvement and stabilization of the lesions without any remarkable toxicity. This case contributes to the growing evidence regarding the efficacy of mTOR inhibitors, such as sirolimus, in multifocal PMH.     

纵隔上皮样肉瘤样血管内皮细胞瘤1例报告

正上皮样肉瘤样血管内皮细胞瘤(epithelioid sarcoma-like hemangioendothelioma,ES-H)是一种血管源性肿瘤,又称假肌源性血管内皮细胞瘤(pseudomyogenic hemangioendothelioma,PH),是上皮样血管内皮细胞瘤(epithelioid heman-gioendothelioma,EH)的亚型~([1])。ES-H多发于青     

假肌源性血管内皮瘤一例分析并文献回顾

目的 探讨假肌源性血管内皮瘤的临床及影像学表现。方法 分析我院一例假肌源性血管内皮瘤的临床表现、实验室检查、影像学及病理学特征,并结合文献进行回顾,总结该病的临床、病理及影像学特征。结果 患者进行实验室及影像学检查后,行右侧髂骨肿物及右臀部皮下肿物切除术,术后恢复良好。结论 假肌源性血管内皮瘤是一种少见的低~中度恶性的血管内皮瘤,影像学上具有软组织肿瘤的特点,熟悉其临床表现、影像学及病理学特征,有助于对该病的诊断及鉴别诊断。     

假肌源性血管内皮瘤一例分析并文献回顾

目的 探讨假肌源性血管内皮瘤的临床及影像学表现。方法 分析我院一例假肌源性血管内皮瘤的临床表现、实验室检查、影像学及病理学特征，并结合文献进行回顾，总结该病的临床、病理及影像学特征。结果 患者进行实验室及影像学检查后，行右侧髂骨肿物及右臀部皮下肿物切除术，术后恢复良好。结论 假肌源性血管内皮瘤是一种少见的低~中度恶性的血管内皮瘤，影像学上具有软组织肿瘤的特点，熟悉其临床表现、影像学及病理学特征，有助于对该病的诊断及鉴别诊断。