Clinical Analysis Of Cutaneous Angiosarcoma Of The Scalp Associated With Kasabach-Merritt Phenomenon

Objective:Cutaneous angiosarcoma of the scalp associated with Kasabach-Merritt phenomenon is a rare soft-tissue sarcoma,and due to the lack of clinical understanding,it led to misdiagnosis or treatment delays.Through the collection of clinical data on cutaneous angiosarcoma of the scalp associated with Kasabach-Merritt phenomenon at home and abroad,the clinical features and treatments were retrospectively analyzed,and COX survival regression model was established to further guide the clinical physicians' understanding and diagnosis of the disease.Methods:The search was conducted on computers,in China Knowledge Network,Wanfang Electronic Periodical Database,PubMed,Cochrane Library,and SinoMed.The key words were "angiosarcoma," "KMP," and "thrombocytopenia." The period is from January 1990.01 to January 2017.Through the collection of relevant domestic and international reports on cutaneous angiosarcoma of the scalp associated with Kasabach-Merritt phenomenon.All data are organized and statistically described using SPSS 15.0 and COX survival regression is used.subsistence analysis.Results:1.A total of 13 cases were included.Among them,except for 1 case lost to follow-up,12 cases had follow-up data,and the outcome was death,including 9 males(70.0%)and 4 females(30.0%).The male to female ratio was 2.2:1;the ages ranged from 43 to 87;the average age was 69.7 years;8 cases(61.5%)occurred in the scalp,3 cases(23.1%)took place on the face,2 cases(15.4%)on torso and limbs;lesions showed mostly purple-red,blue-red patches,a few central ulcers,and satellite nodules on and around the surface,blemishes,ecchymosis,may be accompanied by itching,exudation and pain and discomfort.There were 9 patients(69.2%)with tumors more than 5 cm in diameter and 4 patients(30.8%)with tumors less than 5 cm in diameter.Four patients(30.8%)were diagnosed with KMP before CAS,and nine patients occurred KMP(69.2%)after CAS.In termed of treatments,due to the high degree of malignancy and rapid progress of these diseases,multiple treatments such as surgery,chemoradiation,and glucocorticoids were used.A COX survival regression model was established to evaluate potential treatment factors,“tumor size,” and “order of occurrence of KMP ”.It was determined that on the basis of existing samples,combination therapy could increase the total survival time.of patients in termed of simple treatment.Conclusion:Cutaneous angiosarcoma of the scalp associated with Kasabach-Merritt phenomenon often occurs in the head and face of older men.The skin lesions may present progressively enlarged purple patches with exudation due to the appearance of Kasabach-Merritt phenomenon.When the area is small,surgical resection is feasible,but the local recurrence rate is high,and it is easy to metastasize,and it needs to be combined with other programs to assist treatments.When the area is large and surgery cannot be performed,comprehensive treatments such as radiotherapy and chemotherapy are considered.Later period patients died of DIC,visceral bleeding and infection due to coagulation dysfunction.There is no optimal treatment and further research is needed on this prospective study and and it is determined that on the basis of existing samples,combination therapy can increase the total survival time.of patients in terms of simple treatment.