| Objective: The world's first report of a case of classic Kaposi sarcoma of the Hmong;To analyze the clinical and histopathologic features,the diagnostic and therapeutic methods of Classic kaposi sarcoma.To explore the relationship between virus genotype and phenotype.Methods: One case of classic kaposi sarcoma(CKS)associated with human herpersvirus-8(HHV-8)was reported,in which the dynamic observation of the clinical,pathological and radiological characteristics and the molecular biological identification of the viru and the parallel phylogenetic analysis,the retrospective analysis of the literatures on CKS was demonstrated.Results: A 63-year-old Miao male patient from Southwest China,presented with skin lesion on left eyelid and binaural except arms and legs.He developed skin discoloration followed by nodules that disseminated proximally from the lower extremities to the limbs,ears and the upper left eyelid,following with abdominal discomfort,backache,lower limbs pain,numbness,weakness.Histopathological examination of skin lesions showed that the epidermis was normal,fibrillar blood vessels accumulated in the dermis,spindle cell proliferation,a few mitotic phases,extravasation of red blood cells and deposition of hemosiderin.Immunohistochemistry:CD31(+),CD34(+),D2-40(+),KI67(+),VIM(+).Biopsy and immunohistochemical confirmed the diagnosis of CKS and virology studies of skin lesion confirmed the presence of HHV-8 infection.After 7 years of follow-up observation,it was found that the severity of skin rash and pathological findings were positively correlated with general conditions.Without special treatment,the skin lesions obviously improved before 9 months of follow-up;Viral genotyping was consistent with subtype A based on the amino acid sequence of the open reading frame K1.34 CKS cases were identified.25 were males and 9 females,male to female ratio of 2.8: 1.Geographical distribution: mainly Eurasia,including Turkey(4 cases),Spain(2 cases),Italy(7 cases),Greece(2 cases),Germany(1 case),Korea(1 case),China(11 cases)),Australia(1 case),Brazil(3 cases),Peru(2 cases)also reported individually;Age of onset 36 to 88 years,median age 66.77 years,course of 2 months to 25 years.CKS is characterized by few or widespread multifocal,brown-violescent or dark red patches and papules,plaques and/or deep nodular skin lesions.,often secondary to lymphedema.Lesions: 1 to tens of different.Distribution of skin lesions: limbs or hips(25 cases[73.53%]),trunk(3 case[8.82%]),head and face(7 cases[20.59%]),penis(2 case[5.88%]).Outside the skin damage parts include lymph nodes,conjunctiva,hard palate,lung,liver,spleen,etc.Four of the 34 cases had pain associated with the lesions,four with pruritus.Of the 22 patients with skin infections confined to the skin,3(13.64%)had systemic symptoms,of which 1 had difficulty walking,1 felt a heavy lower limb,and 1 felt a weak lower limb.Of the 5 non-skin infected patients,1(20%)had systemic symptoms associated with shortness of breath,cough,and weight loss.Of 7 patients with disseminated infection,3(42.86%)had systemic symptoms,of which 1 had increased skin temperature,1 had respiratory symptoms,dysphagia,diarrhea,weight loss,and 1 had gastrointestinal symptoms.Skin histopathological examination was performed in 30 of 34 cases,15/34 cases were examined by immunohistochemistry and 4/34 cases were tested for serological virus.15/34 cases(44.12%)received chemotherapy,7/34 cases(23.53%)received radiotherapy and surgical treatment was used in 5 cases(14.71%),3 cases(8.82%)received physiotherapy.Overall,4 cases(11.76%)cured,19 cases(55.88%)improved,9 cases(26.47%)lost their visits.2 patients(5.88%)died related directly to this disease.Conclusion: 1.This patient is the first case of Chinese Hmong CKS report and is also the first report of Guizhou CKS,which opens another perspective for the epidemiological investigation of CKS in China;2.The patient's HHV-8 test belongs to type A,based on phylogenetic studies.Exclusion as a new subtype requires further demonstration;3.In this case,the patient's lesions involving the limbs,eyelids,auricles,gastrointestinal tract and lumbar spine injury do not rule out the association with the disease.Combined with long-term follow-up,it was found that the level of immune status in this patient was related to the development of the disease,and more research was needed to investigate the correlation between them.4.The literature has reported the tendency of CKS to heal spontaneously,so we speculated that maintaining the normal immune system,rather than giving chemotherapy drugs that damage the body's immune system,may play an important role in the outcome of CKS;5.Literature review suggests involvement of CKS areas than the skin,the majority of poor prognosis. |
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