| Objective: Angiosarcoma(AS),also known as malignant vascular endothelial cell tumor,is a type of malignant tumor originating from blood vessels and lymphatic endothelial cells.The incidence is low,accounting for about 1% of soft tissue-derived tumors.At present,there is no unified guide for the treatment of this disease.Seven cases of head and face angiosarcoma were reported,and the related literatures of head and face angiosarcoma from May1997 to March 2019 were retrospectively analyzed to provide clinical experience in the diagnosis of head and face angiosarcoma.Methods: Detailed reports and analysis of 7 cases of rare head and face angiosarcoma,combined with China National Knowledge Infrastructure(CNKI)and other database related literature,analysis and summary of 68 cases of elderly patients with head and face angiosarcoma general statistical data,etiology,clinical manifestations,imaging features,pathology And immunohistochemical features.To explore the diagnosis and treatment of angiosarcoma,and further accumulate the experience of diagnosis and treatment of angiosarcoma.Result: The disease occurs in middle-aged men,7 patients with head and face angiosarcoma,the ratio of male to female is 6:1.The average age of onset was 68.6 years,which was lower than reported in other domestic literature.Farmers,workers engaged in toxic substance testing,and workers exposed to radiation for a long time are prone to the disease.The cause of the disease in Our area is mostly long-term sun exposure,while the domestic literature believes that the disease is related to trauma,hair dyeing,exposure to radiation,contact with toxic chemicals,sun exposure,etc.Patients were treated for head and face nodules and masses.Early clinical manifestations were purple-red,dark red ecchymoses,infiltrative plaques on the head and face,followed by dark red,purple-black nodules with ulcers and hemorrhage.Due to the low incidence of AS,the lesions were not specific at the onset of the disease,Easy to be confused with other diseases,resulting in a longer diagnosis(average 7.3 months).Most patients are misdiagnosed as other diseases,In turn,dermatitis,hemangioma,erysipelas,herpes zoster,scalp hematoma,folliculitis,fungal infection,squamous cell carcinoma and other diseases.so that patients miss the best treatment opportunity.The tumors of 2 patients had metastasized to the lymph nodes,parotid gland,pancreas,liver,bone and other parts at the time of diagnosis.Laboratory and imaging examinations are not specific for diagnosis,but CT,PET/CT,and MRI can be used to understand the metastasis and extent of tumors.When the head and face angiosarcoma patients with platelet reduction and coagulation abnormalities,suggesting a poor prognosis.Diagnosis of head and face AS mainly relies on pathological combined immunohistochemistry.At present,immunohistochemical markers commonly used in AS mainly include CD31,CD34,ERG,Ki-67,Vimentin,and F Ⅷ.In 100% of patients,the intravascular marker CD31 was positive,and 6 cases were positive for CD34(85.7%).CD31 was highly specific when AS was diagnosed.At present,the treatment of head and face AS mainly includes surgery,radiotherapy,chemotherapy,and surgery combined with adjuvant therapy.The main treatment for head and face AS in China is surgery and surgery combined with radiotherapy and chemotherapy.Comparison of domestic and foreign patients: foreign patients are older at the time of diagnosis,but the Chinese patients have a larger tumor area and the incidence of ulcers is higher than abroad.The diagnosis time is late,at which time the risk of metastasis is high and the degree of malignancy is high.Therefore,in terms of treatment,the proportion of early surgery and postoperative radiotherapy abroad is higher than that of China.Conclusion: In areas with strong UV rays,middle-aged and elderly male patients have rapidly increased ecchymotic lesions and blue-violet plaques and nodules on the head and face.When it is not easy to heal using conventional therapy,the possibility of angiosarcoma should be considered.Pathological examination and immunohistochemistry should be performed immediately,which is helpful for early diagnosis and early treatment,and improves the survival rate of patients. |