Kaposi Sarcoma

Cancer-like disease that can be genetically transmitted or acquired, in association with acquired immunodeficiency syndrome (AIDS).

Kaposi Tumor; Kaposi Malignancy; Multiple Idiopathic Pigmented Hemangiosarcoma.

Idiopathic multiple pigment sarcoma described initially in 1872 by the Hungarian dermatologist Moritz Kaposi (1837-1902).

Varies dramatically according to geography. Most common AIDS-associated malignancy in the United States: 73,000-fold more frequent in homosexual HIV-infected men than in the general population; in other HIV-transmission groups, Kaposi sarcoma (KS) affects 1 to 3% of persons other than male homosexuals (however, there is a 10,000-fold increase in HIV-infected women and nonhomosexual men). Two times more frequent in white population men than in black population men.

Rare autosomal dominant forms (rarely familial); mostly acquired (associated with herpes virus 8 infection in AIDS patients, but overexposure to nitrite might be an alternate cause).

Formed by proliferation of abnormal vascular endothelial cells. This has been established by histochemical demonstration of factor VIII-related antigen in both the cells lining the vascular channels and in the spindle-cell component of both classic and AIDS-related Kaposi sarcoma. The tumors appear in a multicentric fashion, although metastatic lesions may develop. The cutaneous distribution is occasionally symmetrical and tends to follow the path of superficial veins.

Lesions have two predominant histologic features: (a) accumulation of spindle cells and (b) presence of vascular elements. The only specific vascular element is the presence of erythrocyte-containing clefts or slits, loosely outlined by tumor cells. Other vascular elements are ectatic capillaries and lymphatics and wide-lumen sinuses.

Classic KS: Multiple red or purple dermal plaques, nodules, and tumors. Lesions occur most commonly on the lower limbs, but may also occur on the upper limbs, trunk, head, neck, and genitalia. Edema is common and hemorrhage of lesions may occur. Involvement of internal organs may occur; in fact, the disease may be exclusively visceral with no cutaneous lesions. The condition is slowly progressive, with death occurring from generalized disease with cachexia, hemorrhages, or impairment of vital organ function by tumor growth.

Endemic KS: Nodular, florid, infiltrative, or lymphadenopathic lesions. The lymphadenopathic type occurs most frequently in children and young adults and may resemble lymphoma.

Epidemic KS: Associated with AIDS. Small, slowly enlarging, rounded tumors distributed over the body. Associated with lymphadenopathy.

Full history of disease progression, including previous anesthesia and present medication. Check complete blood cell count, coagulation status, and serum electrolytes. Assess airway, together with cardiac and respiratory function.

Difficult venous access. Difficulties with tracheal intubation because of involvement of oral mucosa, tonsils, and vocal cords.

Effects of chemotherapy on cardiac and respiratory function.

Bacillary Angiomatosis: This is an infectious capillary proliferation and neutrophilic inflammation caused by the slow-growing gram-negative bacillus Rochalimaea, and difficult to distinguish from KS (skin biopsy is required).

Hemangiomas and Dermatofibroma: Might occasionally and temporarily look like KS, especially in patients with Kast (also called Maffucci) syndrome (enchondroma with multiple angioma that may undergo malignant degeneration, caused by mesodermal dysplasia occurring early in life but with no established genetic transmission).