KAPOSI SARCOMA

Kaposi sarcoma is a spindle-cell tumor

thought to be derived from endothelial
cell lineage.
This condition carries a variable clinical
course ranging from minimal
mucocutaneous disease to extensive
organ involvement
 human herpesvirus type 8 [HHV-8] has
been linked closely with all 4 types of
Kaposi sarcoma.
HHV-8 appears to be necessary to, but
not sufficient for, the development of
Kaposi sarcoma.
immunosuppression appears to be the
most significant cofactor.
 Kaposi sarcoma may be caused by
HHV-8 with stimulation by autocrine and
paracrine growth factors secreted by the
spindle cells themselves as well as the
supporting network of mononuclear and
endothelial cells.
Coinfection with HIV may create a more
aggressive course
 Causes
Coinfection with HIV and HHV-8 (Kaposi
sarcoma-associated herpesvirus [KSHV])
Iatrogenic immunosuppression (including
corticosteroids)
Elevated degree of expression of
numerous cytokines and angiogenic
growth factors, including TNF-alpha, IL-6,
bFGF, HIV-tat protein, and oncostatin M
 Clinical
The lesions may involve:
skin
oral mucosa
lymph nodes
visceral organs.
Most patients present with cutaneous disease.
Visceral disease may occasionally precede
cutaneous manifestations
 Lesions may have macular, papular, nodular,
or plaquelike appearances.
Nearly all lesions are palpable and nonpruritic.
Lesions may range in size from several
millimeters to several centimeters in diameter.
Lesions may assume a brown, pink, red, or
violaceous color and may be difficult to
distinguish in dark-skinned individuals.
Cutaneous lesions may occur at any location but
typically are concentrated on the lower
extremities and the head and neck region.
 Man who is homosexual and has HIV infection and Kaposi sarcoma.
Man who is homosexual and has HIV infection and Kaposi sarcoma.
 Man who is homosexual and has HIV infection and Kaposi sarcoma.
 A 35-year-old man with dome-shaped
locally aggressive tumors, an example
of exophytic Kaposi sarcoma with
cavernous hemangiomalike histology.
 Tumor-associated lymphedema
Typically manifested by lower extremity or
facial involvement, thought to occur
secondary to obstruction of lymphatic
channels
Gastrointestinal lesions can occur
anywhere within the gastrointestinal tract.
Lesions are often asymptomatic and clinically
indolent.
Gastrointestinal disease is usually an indicator of
more advanced HIV infection.
 Pulmonary involvement may be difficult to
distinguish from opportunistic infections
Pulmonary lesions may be an asymptomatic
radiographic finding.
Pleural effusions are often exudative and bloody.
Lymphadenopathy may lead to significant
lymphedema.
may be the only site of disease requiring a
lymph node biopsy.
Unusual varieties of Kaposi sarcoma also exist.
Telangiectatic Kaposi sarcoma is an eruption of
pink translucent nodules with prominent
telangiectasia.
Ecchymotic Kaposi sarcoma appears as periorbital
ecchymoses.
Histologically, there is a large amount of extravasated
red blood cells, a dermis containing foci of
proliferating moderately atypical spindle cells,
vascular slits, erythrophagocytosis, and other features
of Kaposi sarcoma.
On the trunk, lesions often follow skin tension lines.
 Ecchymotic Kaposi sarcoma in a man
who is homosexual.
 Kaposi sarcoma also may arise primarily
in the oral mucosa, lymph nodes, and/or
viscera without skin involvement.
 Epidemic Kaposi sarcoma (KS).
Large violaceous truncal nodules with
typical linear and symmetric
distribution pattern.
 Kaposi sarcoma can be primarily
categorized into:
- epidemic of AIDS-related
- immunocompromised(iatrogenic)
- classic or sporadic
- endemic (African).
 Epidemic AIDS -related Kaposi sarcoma

This entity occurs in patients with advanced HIV

infection and is the most common presentation
of Kaposi sarcoma.
It is the most common malignancy seen in
HIV-infected patients, especially where access
to HAART (highly active antiretroviral therapy) is
limited.
o AIDS-related Kaposi sarcoma is the most
clinically aggressive form of Kaposi sarcoma.
Immunocompromised Kaposi sarcoma

occur following:
- solid-organ transplantation
- patients receiving immunosuppressive
therapy.
The incidence of Kaposi sarcoma is increased
100-fold in transplant patients.
The average time to development of Kaposi
sarcoma following transplantation is 15-30
months.
An aggressive course is the rule with visceral
involvement
Classic (sporadic) Kaposi sarcoma

This entity typically occurs primarily in

elderly men of Mediterranean and
Eastern European background.
It has a male predominance with a male-
to-female ratio of 10-15:1.
The age of onset is between 50 and 70
years
 Classic Kaposi sarcoma usually carries a
protracted and indolent course.
Common complications include venous
stasis and lymphedema.
This form of the disease rarely has lymph
node, mucous membrane, or visceral
involvement.
 Elderly American man of Armenian
origin with characteristic violaceous
plaques of the legs, a good example of
classic Kaposi sarcoma.
 Elderly man of Mediterranean lineage with
hyperkeratotic nodule of Kaposi sarcoma on
sole of foot.
 Endemic African Kaposi sarcoma

This entity occurs primarily in men but

also in women and children who are HIV
seronegative in Africa and may carry an
indolent or aggressive course
The rare wearing of shoes is associated
with an increase of endemic Kaposi
sarcoma (local immune suppression due
to chronic lymphatic obstruction from fine
soil particles)
 Frequency
Approximately 2,500 cases of Kaposi
sarcoma occur yearly in the United States
at the present time.
An aggressive form of Kaposi sarcoma
began to appear among homosexual men
In the United States, the risk of Kaposi
sarcoma among sexually active
homosexual men is much greater than
among others infected with HIV
 The other major group in the United States in whom Kaposi sarcoma
occurs is the posttransplant population, in whom the incidence is
about 1 in 200.

Prior to the advent of HIV, Kaposi sarcoma was common in

central Africa and prevalent in Mediterranean countries and the
Middle East

In Africa, the incidence of Kaposi sarcoma is very high at 37.7

per 100,000 in men and 20.5 per 100,000 in women.

In Europe, the highest rates of classic Kaposi sarcoma are in

Sicily (Ragusa, 30.1 cases per million in men/5.4 cases per
million in women) and Sardinia (24.3 cases per million in
men/7.7 cases per million in women).
 Kaposi Sarcoma:
Differential Diagnoses
Bacillary Angiomatosis
Blue Rubber Bleb Nevus Syndrome
Nevi, Melanocytic
Pyogenic Granuloma (Lobular Capillary
Hemangioma)
Tufted Angioma
 Other Problems to Be
Considered
Melanoma
Cavernous hemangioma
Angiokeratoma
Carcinoma cutis (especially renal cell
carcinoma)
Nodal myofibromatoma
Arteriovenous malformations (pseudo-Kaposi
sarcoma)
Severe statis dermatitis (pseudo-Kaposi
sarcoma)
Reactive angioendotheliomatosis
 Laboratory Studies
Immunohistochemical detection of
human herpes virus-8 latent nuclear
antigen-1 has been claimed as useful in
the diagnosis of Kaposi sarcoma.
Assays for HHV-8 have been challenging.
At present, no universally accepted
method exists.
Methods based on both lytic and latent-
phase viral antigens remain promising.
 Serum glucose levels may reflect an increased
incidence of diabetes mellitus in patients with
classic Kaposi sarcoma (KS).
Eosinophilia is seen especially in African
patients and patients who are homosexual, in
whom parasitosis may be common.
In KS-AIDS, cytopenia of 1 or more cell lines is
frequent.
 Imaging Studies
Computed tomography (CT) may be
valuable, especially abdominal CT scans
in patients with AIDS
o In AIDS-related Kaposi sarcoma, early
lymphatic and hepatosplenic involvement may
be evident.
Radionucleotide scans may be useful in
demonstrating visceral Kaposi sarcoma
and associated lymphoma.
 In both adults and children with pulmonary
Kaposi sarcoma, chest radiography
shows perihilar and lower zone
involvement.
Pleural effusions appear to be more
common on radiographs in children.
With lung nodules, the distinction between
Kaposi sarcoma, lymphoma, and/or
opportunistic infection may be challenging.
 Evaluation for Kaposi sarcoma should
include a complete physical examination
and a biopsy of suspected lesions
including lymph nodes
Histologic Findings tends to demonstrate
increased spindle cells with vascular
slits and vascular structures with a
predominance of endothelial cells.
 Staging
Stage I - Localized nodular Kaposi sarcoma in
elderly men in North America and Europe
Stage II - Localized, invasive, and aggressive
Kaposi sarcoma (mostly seen in Africa)
Stage III - Disseminated mucocutaneous
Kaposi sarcoma in African children and
patients who are homosexual
Stage IV - Stage III with visceral involvement
 Medical Care
Treatment usually is based on the extent of
disease and the patient's immune status
Management modalities for Kaposi sarcoma
include nonintervention, surgical removal of skin
nodules or severely affected areas (eg, areas of
the extremities, intussuscepted bowel), laser
surgery, conventional and megavoltage
radiotherapy, chemotherapy, immunotherapy,
antiviral drugs, and cessation of
immunosuppressive therapy in iatrogenically
immunosuppressed patients.
 Localized nodular disease may respond
well to:
surgical excision
Radiotherapy
intralesional and outpatient low-dose
vinblastine chemotherapy.
 Laser therapy: Argon laser
photocoagulation therapy also may be
beneficial in classic Kaposi sarcoma
lesions.
Solitary Kaposi sarcoma lesions may be
excised surgically or removed using laser
surgery.
 Medication
Treatment of classic Kaposi sarcoma
may also be accomplished with topical
imiquimod.
intralesional injections of vinblastine for
persistent cutaneous nodules and
intraarterial vinblastine in certain settings
for locally aggressive Kaposi sarcoma
(KS).
 Systemic vinblastine (3.5-10 mg IV
weekly with, at times, 1 intralesional
injection of 0.1 mg) usually is best for both
the patient with classic Kaposi sarcoma
and, occasionally, patients with KS-AIDS.
Drugs with proven efficiency include
vincristine, vinblastine, dacarbazine,
doxorubicin, and actinomycin D.
 The combination of zidovudine, interferon, and
low-dose intravenous vinblastine may be used
for patients with KS-AIDS.
In iatrogenic Kaposi sarcoma, cessation of
immunosuppressive therapy may be the most
effective treatment.
Sirolimus has the advantage of decreased risk
of malignancies, including Kaposi sarcoma,
associated with its use compared with other
immunosuppressants, namely calcineurin
inhibitors, and possibly Kaposi sarcoma
regression