Kaposi sarcoma

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sarcoma

 [sahr-ko´mah] (pl. sarcomas, sarco´mata.)
A tumor, often highly malignant, composed of cells derived from connective tissue such as bone, cartilage, muscle, blood vessel, or lymphoid tissue; sarcomas usually develop rapidly and metastasize through the lymph channels. Different types are named for the specific tissue they affect: fibrosarcoma in fibrous connective tissue; lymphosarcoma in lymphoid tissues; osteosarcoma in bone; chondrosarcoma in cartilage; rhabdosarcoma in muscle; and liposarcoma in fat cells. adj., adj sarco´matous.
Abernethy's sarcoma a malignant fatty tumor occurring mainly on the trunk.
alveolar soft part sarcoma one with a reticulated fibrous stroma enclosing groups of sarcoma cells enclosed in alveoli walled with connective tissue.
botryoid sarcoma (sarcoma botryoi´des) an embryonal rhabdomyosarcoma arising in submucosal tissue, usually in the upper vagina, cervix uteri, or bladder neck in young children and infants, presenting grossly as a polypoid grapelike structure.
endometrial stromal sarcoma a pale, polypoid, fleshy, malignant tumor of the endometrial stroma.
Ewing's sarcoma a malignant tumor of the bone that arises in medullary tissue, usually found in cylindrical bones, with pain, fever, and leukocytosis as prominent symptoms; called also Ewing's tumor.
giant cell sarcoma a malignant form of giant cell tumor of bone.
granulocytic sarcoma chloroma.
immunoblastic sarcoma of B cells an aggressive B-cell lymphoma believed to arise from transformed interfollicular B lymphocytes, which in many cases is associated with a preexisting immunologic disorder, e.g., Sjögren's syndrome, systemic lupus erythematosus, or Hashimoto's thyroiditis, or with an immunocompromised state.
immunoblastic sarcoma of T cells a group of T-cell lymphomas comprising tumors derived from T lymphocytes in the paracortical area arising from a mixture of small lymphocytes and many large transformed cells; the latter are characterized by one or more small but distinctly eosinophilic nucleoli.
Kaposi sarcoma a multicentric, malignant neoplastic vascular proliferation characterized by bluish red cutaneous nodules, usually on the lower extremities, most often on the toes or feet; the nodules slowly increase in size and number and spread to more proximal sites. Tumors often remain confined to skin and subcutaneous tissue, but widespread visceral involvement may occur. The condition occurs endemically in certain parts of Central Africa and Central and Eastern Europe, and a particularly virulent and disseminated form occurs in immunocompromised patients such as transplant recipients and those with acquired immunodeficiency syndrome. Human herpesvirus 8 has been implicated as a causative agent.
lymphatic sarcoma diffuse lymphoma.
osteogenic sarcoma a malignant primary tumor of bone composed of a malignant connective tissue stroma with evidence of osteoid, bone, and/or cartilage formation; depending upon the dominant component, classified as osteoblastic, fibroblastic, or chondroblastic.
pseudo–Kaposi sarcoma unilateral subacute to chronic dermatitis, often with postinflammatory hyperpigmentation, occurring in association with underlying arteriovenous fistula, which closely resembles Kaposi sarcoma both clinically and histologically.
reticulum cell sarcoma histiocytic lymphoma.

Ka·po·si sar·co·ma (KS),

(kă-pō'zē), [MIM*148000]
a multifocal malignant neoplasm of primitive vasoformative tissue, occurring in the skin and sometimes in the lymph nodes or viscera, consisting of spindle cells and irregular small vascular spaces frequently infiltrated by hemosiderin-pigmented macrophages and extravasated red blood cells. Clinically manifested by cutaneous lesions consisting of reddish-purple to dark-blue macules, plaques, or nodules; seen most commonly in men older than 60 years of age and in AIDS patients, as an opportunistic disease associated with human herpes virus-8 infection.

Kaposi sarcoma

A once rare, indolent malignancy that affected older European men in the pre-AIDS era. In the AIDS era, Kaposi sarcoma (KS) often develops in homosexual men infected with human herpesvirus 8 and in those immunocompromised by transplantation, immunosuppression or lymphoproliferation. KS is characterised by a proliferation of lymphatic or vascular channels, driven by growth and regulatory factors—e.g., IL-1-beta, IL-6 and tat protein.

Clinical forms
Classically—elderly Italian or Jewish men; AIDS related—homosexual men.
 
DiffDx
Bacillary angiomatosis.

Management
If lesion is single, surgical excision or 8–12 Gy radiation therapy (85% respond), or intralesional IFN-alpha; if multiple, chemotherapy (e.g., doxorubicin, bleomycin, vincristine); multimodality therapy.
 
Prevention
Antivirals—e.g., ganciclovir, foscarnet, cidofovir, adefovir.

Kaposi sarcoma

AIDS A once rare, indolent malignancy affecting older Italian or Jewish ♂; in the AIDS era, KS develops in homosexual ♂ infected with human herpesvirus 8; in those immunocompromised by transplantation, immunosuppression or lymphoproliferation; KS is characterized by a proliferation of lymphatic or vascular channels, driven by growth and regulatory factors–eg, IL-1-β, IL-6 and tat protein Clinical forms Classic–elderly European ♂; AIDS related, homosexual ♂ Treatment If lesion is single, surgical excision or 8–12 Gy RT–85% respond, or intralesional IFN-α; if multiple, chemotherapy–eg, doxorubicin, bleomycin, vincristine; multimodality therapy Prevention Antivirals–eg, ganciclovir, foscarnet, cidofovir, adefovir. See AIDS, Anti-LANA antibody, Human herpesvirus 8, Promontory sign.

Ka·po·si sar·co·ma

(KS) (kap'ŏ-shē sahr-kō'mă)
A multifocal malignant neoplasm of primitive vasoformative tissue, occurring in the skin and sometimes in lymph nodes or viscera, consisting of spindle cells and irregular small vascular spaces frequently infiltrated by hemosiderin-pigmented macrophages and extravasated red blood cells; clinically manifested by cutaneous lesions consisting of reddish-purple to dark-blue macules, plaques, or nodules; seen most commonly in men older than 60 years of age and, in AIDS patients, as an opportunistic disease associated with human herpes virus 8 infection.
Enlarge picture
KAPOSI'S SARCOMA: on skin

Kaposi sarcoma

Abbreviation: KS
A lymphatic endothelial malignancy, rather than a true sarcoma, composed of multiple red or purple macules, papules, or nodules, that is first apparent on the skin or mucous membranes but may involve the internal organs. Once a rare disease seen primarily in elderly men of Mediterranean, African, or Ashkenazi descent (so-called classic KS), it is the most common cancer related to AIDS. In patients with AIDS, KS is believed to be sexually acquired due to acquisition of human herpesvirus 8. When KS is associated with AIDS, it progresses and disseminates rapidly to multiple skin sites, as well as the lymph nodes and visceral organs. See: illustration; AIDS

Symptoms

The lesions are typically painless but may be cosmetically disfiguring or may interfere with internal organ function. They are found most often on the dorsa of the feet and lower extremities in patients with classic KS, and on the face, trunk, oral cavity, and internal organs in immunosuppressed patients. KS is sometimes referred to as “epidemic” in patients with HIV infection; “endemic” in parts of Africa; and “acquired” in patients taking immune-suppressing drugs after organ transplantation. In advanced disease, the lesions may merge into large plaques, sometimes blocking lymphatics and causing localized edema. Involvement of internal organs, primarily the gastrointestinal tract or the lungs, may result in dyspepsia or dyspnea.

Diagnosis

Characteristic tumors on the skin suggest the diagnosis, which should be confirmed by tissue biopsy.

Treatment

Treatment options include radiation therapy, cancer chemotherapies, cryotherapy, hormone therapies, and biotherapy (interferon alfa-2b).

Patient care

Epidemic KS may profoundly alter the patient's appearance. Emotional support for the patient and family may help them cope with the diagnosis and its effects on body image. Psychological counseling may be needed. Standard precautions should be followed when assessing or caring for the patient. The skin should be assessed for new lesions at each health care contact.

Kaposi,

Moritz (born Moritz Kohn), Hungarian dermatologist in Austria, 1837-1902.
Kaposi sarcoma - a multifocal malignant neoplasm. Synonym(s): multiple idiopathic hemorrhagic sarcoma
Kaposi varicelliform eruption - a rare complication of vaccinia superimposed on atopic dermatitis, with generalized vesicles and papulovesicles and high fever. Synonym(s): eczema vaccinatum

Ka·po·si sar·co·ma

(KS) (kap'ŏ-zē sahr-kō'mă) [MIM*148000]
Multifocal malignant neoplasm of primitive vasoformative tissue, occurring in skin and sometimes lymph nodes or viscera. Clinically manifested by cutaneous lesions consisting of reddish-purple to dark-blue macules, plaques, or nodules; seen most commonly in men older than 60 years of age and in AIDS patients.
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