Kaposi’s sarcoma is associated with
Correct Answer: HIV infection
Description: Ans. a (HIV infection). (Ref. Robbins, Pathology, 7th ed., 256)KAPOSI'S SARCOMA (KS)# Kaposi's sarcoma, a highly vascular tumor is most common neoplasm in AIDS. Other neoplasms found in HIV patients are: B-cell NHL, primary brain lymphoma and invasive carcinoma cervix.# HHV-8 or KSHV is strongly implicated as a viral cofactor in the pathogenesis of KS.# Cutaneous lesions are the most common.# Factor VIII of coagulation cascade is immunohistochemistry marker of KS.# Clinically, the initial lesion may be a small, raised reddish-purple nodule on the skin, a discoloration on the oral mucosa, or a swollen lymph node.# 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).# Some 80% of patients with pulmonary KS have cutaneous lesions, which appear as raised macules.# Apart from skin, lymph nodes, gastrointestinal tract, and lungs are the organ systems most commonly affected by KS.# Lesions have been reported in virtually every organ, including the heart and the CNS.# 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 KS and is of no special clinical significance.# CXR characteristically shows bilateral ground-glass haziness, lower lobe infiltrates that obscure the margins of the mediastinum and diaphragm.# Pleural effusions are seen in 70% of cases of pulmonary KS, a fact that is often helpful in the differential diagnosis.# A diagnosis of KS is based upon biopsy of a suspicious lesion.# Histologically one sees a proliferation of spindle cells and endothelial cells, extravasation of red blood cells, hemosiderin-laden macrophages, and, in early cases, an inflammatory cell infiltrate.Management of KS:# Spontaneous regressions have been reported in the setting of HAART.# Treatment is indicated under two main circumstances. The first is when a single lesion or a limited number of lesions are causing significant discomfort or cosmetic problems, such as with prominent facial lesions, lesions overlying a joint, or lesions in the oropharynx that interfere with swallowing or breathing.# Under these circumstances, Rx with localized radiation, intralesional vinblastine, or cryotherapy may be indicated.# Note that patients with HIV infection are particularly sensitive to side effects of radiation therapy.# The single most important determinant of response appears to be the CD4 T cell count.# IFN-alpha provides an added advantage of having antiretroviral activity; thus, it may be the appropriate first choice for single-agent systemic therapy for early patients with disseminated disease.# Liposomal daunorubicin, liposomal doxorubicin, and paclitaxel have been approved by the FDA for this indication. Liposomal daunorubicin is approved as first-line therapy for patients with advanced KS.
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