All are true regarding kaposi sarcoma except one :
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Correct Answer:
Occurs in AIDS pt only
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Ans. is 'd' ie Occurs in AIDS pt. only There are at least 4 distinct epidemiological forms of Kaposi's sarcoma, only one form is associated with AIDS.The 4 distinct forms are -The classic form - that occurs in older men of predominantly Mediterranean or Eastern European, Jewish backgrounds.Equatorial African form - that occurs in all ages.The form associated with organ transplantationThe form associated with HIV-I infection.Kaposi 's sarcoma (K.S.)Kaposi's sarcoma is a multicentric neoplasm consisting of multiple vascular nodules appearing in the skin, mucous memb and lymph nodes, lung, GIT etc.Lesions often appear in sun-exposed areas, particularly the tip of the nose, and have a propensity to occur in areas of trauma (Koebner phenomenon)Course ranges from indolent with only minor skin or lymph node involvement to fulminant with extensive cutaneous and visceral involvement.AIDS related Kaposi's sarcoma occurs predominantly in homosexual men (96%)K.S. does not result from a neoplastic transformation of cells in the classic sense and so is not truly a sarcoma. It is a manifestation of excessive proliferation of spindle cells that are believed to be of vascular origin, it is more a consequence of disordered cytokine regulation of cell growth than a true cancer. Generally speaking the tumor respects tissue planes and is rarely invasive. Disseminated K.S. is believed to be d/t occurrence of multiple neoplasm all over the body rather than to metastasis.Though K.S. is an opportunistic ds in HIV-infected individuals but unlike opportunistic infection, its occurrence is not strictly related to the level of depression of CD4+T cells counts. It may be seen at any stage of HIV inf. even in the presence of normal CD4+T cell count.Human Herpes virus-8 (HHV-8) is universally present in all forms of KS (in all the 4 types)In contrast to most malignancies, in which lymph node involvement implies metastatic spread and a poor prognosis, lymph node involvement may be seen very early in K.S. and is of no clinical significance.Management of KS associated with HIVIn the majority of cases, effective ARV therapy is quite helpful in achieving control. Spontaneous regressions have been reported in the setting of HAART.For patients in whom tumor persists or in whom control of HIV replication is not possible options exist.Treatment is indicated under two main circumstances.For a single lesion or a limited number of lesions causing significant discomfort or cosmetic problemslocalized radiation,intralesional vinblastine, orcryotherapyFor large number of lesions or visceral involvementSystemic therapies, either IFN- or chemotherapy is consideredInterferon- IFN provides an added advantage of having ARV activity; thus, it may be the appropriate first choice for single-agent systemic therapy for early patients with disseminated disease.Chemotherapy- Three of the chemotherapeutic agent that have been approved by the FDA for KS are:liposomal daunorubicin,liposomal doxorubicin, andpaclitaxelLiposomal daunorubicin is approved as first-line therapy for patients with advanced KS.The single most important determinant of response appears to be the CD4+ T cell count.Management of AIDS Associated Kaposi's SarcomaAntiretroviral therapySingle or limited number of lesionsLocalized radiationIntralesional vinblastineCryotherapyExtensive diseaseInitial therapyInterferon- (if CD4+ T cells >150/L)Liposomal daunorubicinSubsequent therapyLiposomal doxorubicinPaclitaxelCombination chemotherapy with low-dose doxorubicin, bleomycin, and vinblastine (ABV)Radiation treatment So the option (C) is not totally correct but option (D) is absolutely wrong.
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