Pdf pathology of rituximabinduced kaposi sarcoma flare. Kaposi sarcoma ks is an angioproliferative disorder that requires infection with human herpes virus 8 hhv8, also known as kaposi sarcomaassociated herpes virus kshv, for its development. From the department of pathology, university of pittsburgh. The tumors lesions of kaposis sarc oma typically appear as painless purplish spots on the legs, feet or face. Ks generally occurs in people with weakened immune systems. They are made of cancer cells, blood vessels, and blood cells. The 1994 discovery of kaposis sarcomaassociated herpesvirus kshv, human herpesvirus 8, hhv8 in kaposis sarcoma ks tissues had a huge impact, not only in the field of virology, but also on bioscience generally chang et al. The exact mechanism responsible for iatrogenic ks flare is unclear. The differential diagnosis of kaposi sarcoma includes many disease that range from benign. Pathology of rituximabinduced kaposi sarcoma flare bmc. Kaposis sarcoma ks is the most common tumor in hivinfected individuals in africa 1.
Kaposi sarcoma pathogenesis translational biomedicine. Kaposi sarcoma ks was first described by the viennese dermatologist moritz kaposi 1 18371902 more than a century ago. Learn more about the different types, causes, symptoms, diagnosis, treatment, and prevention of kaposis sarcoma. Kaposi sarcoma ks is a lowgrade vascular tumor that is rare among the general us population, with an incidence rate of less than 1 per 100,000.
Kaposi sarcoma is a cancer that causes lesions abnormal tissue to grow in the skin. It usually appears as purple, red, or brown blotches or tumors on the skin, or on mucosal surfaces such as inside the mouth. The low power view of the histology of kaposi sarcoma is of a cellular dermal nodule figure 1. However, it was relatively common in south africa before the hivaids. Kaposis sarcoma synonyms, kaposis sarcoma pronunciation, kaposis sarcoma translation, english dictionary definition of kaposis sarcoma. It was described in 1872 by the hungarian dermatologist mortiz kaposi, and was an isolated and sporadic occurrence before the emergence of hiv infection and aids.
However, little information is available about the immunohistochemical characteristics of kaposi sarcoma. Aids associated tumors with anaplastic histology appear to have a. However, the majority of ks cases occur when infected patients also suffer from some coincident form of immune deregulation, providing a favorable. Immunohistochemical characteristics of kaposi sarcoma and its. Acquired immune deficiency syndrome aids is a disease of the immune system caused by infection with the human immunodeficiency virus hiv. Find out why people with hiv are more prone to developing the cancer. Kaposi sarcoma in a patient with postpolio syndrome. Some people with several risk factors never develop. Ks a cancer characterized by numerous bluishred nodules on the skin, usually on the lower extremities, that is endemic to equatorial africa and. The features change depending on the nature of the lesion clinically as it progresses from the patch to plaque to nodular phase histology of kaposi sarcoma. It was the first malignancy to be linked with acquired immunodeficiency syndrome aids. The disease is multifocal, with a course ranging from indolent, with only skin manifestations to fulminant, with extensive visceral involvement. Kaposis sarc oma is a type of cancer that forms in the lining of blood and lymph vessels.
Head and neck kaposi sarcoma lester dr thompson, md. Kaposi sarcoma ks is a lowgrade vascular tumor associated with infection with human herpesvirus 8 hhv8, also known as the ksassociated herpesvirus kshv. Kaposis sarcoma definition of kaposis sarcoma by the. The lesions are usually purple and are made of cancer cells, new blood vessels, red blood. Benign lymphangioendothelioma acquired progressive lymphangioma. Kaposi sarcoma ks is a softtissue tumor that arises in people infected with the human herpes virus8 hhv8. Kaposi sarcoma with laryngeal, tracheal, and parenchymal. Pathology of rituximabinduced kaposi sarcoma flare article pdf available in bmc clinical pathology 81. Its sudden leap to prominence has increased interest in understanding the tumor. Pdf histological variants of cutaneous kaposi sarcoma. Kaposis sarcoma ks is a type of cancer that people with aids often get. This enigmatic vascular neoplasm has since received much attention in the literature, especially after recognition of its association with the acquired immune deficiency syndrome aids in the early 1980s.
Kaposi sarcoma is an unusual vascular neoplasm that forms abnormal, slitlike vascular spaces containing compressed red cells see fig. King md, phd, in elseviers integrated pathology, 2007. Kaposi sarcoma is grouped within vascular tumours of variable malignant behaviour. Kaposi sarcoma ks is a disease of the endothelial cells of blood vessels and the lymphatic system. The tumors lesions of kaposis sarcoma typically appear as painless purplish spots on the legs, feet or face. Kaposis sarcoma is mostly seen in people with an advanced hiv infection it can also affect people who have a weakened immune system for another reason, such as after having an organ transplant. Kaposis sarcoma ks, an angioproliferative disorder, has a viral etiology and a multifactorial pathogenesis hinged on an immune dysfunction. Get detailed information about the diagnosis and treatment of newly diagnosed and recurrent kaposi sarcoma in this summary for clinicians. It commonly appears in multiple locations on the skin and around one or more the following areas. Before the discovery of kshv, almost all viruses had been identified using conventional virus isolation methods with cell cultures. Kaposi sarcoma ks tends to demonstrate increased spindle cells with. Kaposi sarcoma ks develops from the cells that line lymph or blood vessels.
Lesions may also develop in the digestive tract, liver, or lungs. Kaposi sarcoma ks causes red or purple patches of tissue lesions to grow under the skin and in the lining of the mouth, nose, and throat. In severe kaposis sarc oma, lesions may develop in the digestive tract and lungs. Lesions can also appear in the genital area, mouth or lymph nodes. Despite its name, it is no longer classified as a sarcoma which is a malignant tumour of mesenchymal origin as it is due to multicentric vascular hyperplasia. Kaposis sarcoma, a rare tumor previously known mostly to dermatologists, pathologists, and oncologists, has become almost commonplace since the onset of the acquired immunodeficiency syndrome aids in the early 1980s. Human herpesvirus 8 and iron staining are useful in differentiating.
Kaposi sarcoma ks gained public attention as an aidsdefining malignancy. The differential diagnosis of kaposi sarcoma includes many disease that range from benign disease to malignant tumors. These patches, or lesions, are usually red or purple. Kaposi sarcoma was long recognized as an indolent tumor occurring on the lower extremities of elderly men of eastern mediterranean origin. Kaposis sarcoma is a rare type of cancer caused by a virus. You will find out more about the factors that increase the chance of developing kaposi sarcoma. Trends and histopathological patterns of kaposi sarcoma at the. Kaposi sarcoma is a rare tumor that occurs due to a type of herpesvirus. Aidsassociated tumors with anaplastic histology appear to have a. Since kaposi 1872 described the multiple pig mented haemorrhagic sarcoma of the skin which bears his. But ks can also develop in other parts of the body, such. Kaposi s sarcoma ks is a vascular tumor that was brought to the attention of the medical community over a century ago in a fascinating case series in which purplecoloured nodular skin lesions were observed on. Kaposi sarcoma ks is characterized by angioproliferative multifocal tumors of the skin, mucosa and less frequently the viscera. Anatomic pathology kaposi sarcoma vs granuloma annulare.
Kaposi sarcoma ks is a complex cancer that arises from the initial infection of an appropriate endothelial or progenitor cell by kaposi sarcoma herpesvirushuman herpesvirus8 kshvhhv8. They can occur singularly, in a limited area, or be widespread. Some had coexistent pathology, mainly superimposed candidiasis, but also a few had. Request pdf on jun 1, 2009, liron pantanowitz and others published pathology of kaposis sarcoma find, read and cite all the research you need on researchgate. In severe kaposis sarcoma, lesions may develop in the digestive tract and lungs.
Uncommon, low grade, vascular malignancy caused by kaposi sarcoma herpesvirus human herpesvirus 8 kshv hhv8 infection arch pathol lab med 20. Histological variants of cutaneous kaposi sarcoma ncbi. The 1994 discovery of kaposis sarcoma associated herpesvirus kshv, human herpesvirus 8, hhv8 in kaposi s sarcoma ks tissues had a huge impact, not only in the field of virology, but also on bioscience generally chang et al. Kaposi sarcoma is a disease in which malignant lesions cancer can form in the skin, mucous membranes, lymph nodes, and other organs. A close inspection of ks lesions reveals that they are largely comprised of cells of endothelial origin with a unique spindled morphology, and these are accompanied by a variable chronic inflammatory infiltrate. Classic mediterranean kaposi sarcoma ks m f, 40 70 years, mediterranean or ashkenazi origin. Stein me, spencer d, ruff p, lakier r, macphail p, bezwoda wr. Pathology of kaposis sarcomaassociated herpesvirus infection. Kaposi sarcoma memorial sloan kettering cancer center. Kaposis sarcoma ks is a vascular tumor that was brought to the attention of the medical community over a century ago in a fascinating case series in which purplecoloured nodular skin lesions were observed on. Slitlike vascular spaces formed by spindled endothelial cells with minimal to moderate atypia. Human herpesvirus 8 hhv8 is found in the lesions of all those who are affected. It affects the skin and mouth, and sometimes the internal organs. Review article from the new england journal of medicine kaposis sarcoma.
A risk factor is anything that increases a persons chance of developing cancer. Which histologic findings are characteristic of kaposi sarcoma ks. Kaposi sarcoma is an aidsdefining illness associated with human herpes virus8 hhv8 coinfection. Liron pantanowitz, md, department of pathology, university of pittsburgh medical center, upmc shadyside, upmc cancer pavilion suite 201, 5150 centre ave, pittsburgh, pa 15232 email. Although risk factors often influence the development of cancer, most do not directly cause cancer. The features change depending on the nature of the lesion clinically as it progresses from the patch to plaque to nodular phase. The clinical presentation of ks varies from indolent skin lesions to extensive visceral disease.
Histological variants of cutaneous kaposi sarcoma diagnostic. Kaposi sarcoma ks flare may occur following therapy with corticosteroids, as part of the immune reconstitution inflammatory syndrome seen with highly active antiretroviral therapy haart, and after rituximab therapy. Its prevalence and aggressiveness have decreased since antiretroviral therapy has become widespread, especially in developed countries. In people with hiv, ks is an aidsdefining condition. It begins in the cells that form blood or lymph vessels and first appears as distinctive deep purple or reddish blue lesions on the skin. Kaposi sarcoma in people with hivaids is often called epidemic kaposi sarcoma. Kaposi sarcoma treatment depends upon the type and can include curettage and desiccation, radiation therapy, surgery, and sometimes chemotherapy. A case of aidsassociated cutaneous ks flare following rituximab therapy was compared to similar controls. We report a case of kaposis sarcoma in a newly diagnosed retroviral homosexual patient with cd4 count of 21.
Metastatic kaposi sarcoma with osseous involvement in a. Kaposi sarcoma ks is the most common malignancy associated with human immunodeficiency virus hiv infection. Kaposi sarcoma ks is a lowgrade vascular tumor associated with kaposi. Immunohistochemical characteristics of kaposi sarcoma and. Ks is classified into four types based on the clinical circumstances in which it develops.