Resident Short Review
Kaposi Sarcoma
Oana Radu, MD; Liron Pantanowitz, MD
 Kaposi sarcoma (KS) is a low-grade vascular tumor
associated with Kaposi sarcoma herpesvirus/human her-
pesvirus 8 (KSHV/HHV8) infection. Kaposi sarcoma lesions
predominantly present at mucocutaneous sites, but may
involve all organs and anatomic locations. Recognized
epidemiologic-clinical forms of KS include classic, African
(endemic), AIDS-associated (epidemic), and iatrogenic KS.
New clinical manifestations have been described, such as
antiretroviral therapyrelated KS regression or flares.
Kaposi sarcoma lesions evolve from early (patch stage)
macules into plaques (plaque stage) that grow into larger
nodules (tumor stage). Newer histologic variants include
K aposi sarcoma (KS) was first described by the Viennese
dermatologist Moritz Kaposi1 (18371902) more than a
century ago. 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. The etiologic
agent Kaposi sarcoma herpesvirus/human herpesvirus 8
(KSHV/HHV8) was identified in KS lesions by Chang et al2
in 1994. This triggered the accrual of much more informa-
tion about KS, particularly as KS is used as a model to study
various aspects of carcinogenesis, including viral oncogen-
esis, angiogenesis, and cancer immunology. Worldwide
HHV8 seropositivity exceeds the incidence of KS, suggest-
ing that other cofactors (eg, blood-sucking arthropods and
iron) may be implicated in KS development.
Kaposi sarcoma is a low-grade vascular tumor that may
involve the skin, mucosa, and viscera, developing in 1 of 4
different epidemiologic-clinical settings.3 In recent years
there have been several changes in our understanding of
KS, including its evolving epidemiology, pathogenesis, new
clinical presentations and associations, descriptions of new
histologic variants, and the emergence of novel biomarkers
with promising targeted therapeutic agents.4,5 Despite these
advances, KS remains the most prevalent malignancy
among patients with AIDS. Moreover, KS continues to
plague patients with drug-related or transplant-associated
immunosuppression. Hence, it is essential that pathologists
Accepted for publication April 27, 2012.
From the Department of Pathology, University of Pittsburgh
Medical Center, Pittsburgh, Pennsylvania.
The authors have no relevant financial interest in the products or
companies described in this article.
Reprints: Liron Pantanowitz, MD, Department of Pathology,
University of Pittsburgh Medical Center, UPMC Shadyside, UPMC
Cancer Pavilion Suite 201, 5150 Centre Ave, Pittsburgh, PA 15232
(e-mail: pantanowitzl@upmc.edu).
Arch Pathol Lab MedVol 137, February 2013
anaplastic, hyperkeratotic, lymphangioma-like, bullous,
telangiectatic, ecchymotic, keloidal, pyogenic granulo-
malike, micronodular, intravascular, glomeruloid and
pigmented KS, as well as KS with sarcoidlike granulomas
and KS with myoid nodules. Latency-associated nuclear
antigen (HHV8) is the most specific immunohistochemical
marker available to help distinguish KS from its mimics.
Since KS remains one of the most common AIDS-defining
malignancies, it is important that pathologists be able to
recognize KS and its contemporary manifestations.
(Arch Pathol Lab Med. 2013;137:289294; doi: 10.5858/
arpa.2012-0101-RS)
be able to recognize KS and its contemporary manifesta-
tions.
PATHOGENESIS
With the advent of genomic technologies, we now know
that proliferating KS spindle tumor cells are of endothelial
origin, confirming former studies that used histochemistry
and ultrastructural findings. Circulating blood mononuclear
and endothelial progenitor cells are believed to be the
source of early KS lesions. Infection with HHV8 reprograms
the hosts blood endothelial cells so that they resemble
lymphatic endothelium, upregulating several lymphatic-
associated genes such as lymphatic vessel endothelial
receptor 1 (LYVE1), podoplanin, and vascular endothelial
growth factor receptor 3 (VEGFR3).6 However, HHV8
infection alone appears to be insufficient for the develop-
ment of KS. Kaposi sarcoma progression relies also on some
degree of host immune dysfunction and the local inflam-
matory milieu.7,8 Kaposi sarcoma growth involves the
upregulation of many key HHV8 gene products, such as
the latency-associated nuclear antigen (LANA-1 or LNA-1).
Like other herpesviruses, HHV8 remains latent within cells
and has developed a variety of mechanisms to evade the
host immune system. The growing understanding of these
and other molecular events involved in KS development has
fueled a number of clinical trials using novel therapeutic
agents.9
CLINICAL FEATURES
The 4 recognized epidemiologic-clinical forms include
classic, African (endemic), AIDS-associated (epidemic), and
iatrogenic (or transplant-associated) KS (Table). Patients of
any age may develop KS, even newborns. In general, there is
a male predilection. The lack of sex hormone receptors on KS
tumor cells argues against a direct effect of sex hormones and
suggests that other unknown factors may be involved in this
sex bias. Human immunodeficiency virus (HIV)related KS in
Kaposi SarcomaRadu & Pantanowitz 289
 Different Epidemiologic-Clinical Forms of Kaposi Sarcoma (KS)
KS Type Epidemiology
Classical Mainly males aged 4070 years, of
Mediterranean or Jewish Ashkenazi
origin
African Middle-aged black adults and
children from equatorial Africa
AIDS-associated Mainly homosexual males and
intravenous drug users aged 2050
years; now equally affects women
and children in Africa
Iatrogenic Immunosuppressed persons of any
age from autoimmune disease,
drugs, or transplantation
Clinical Distribution
Skin of the lower extremities, but
mucosal and visceral lesions may
develop
Multiple localized skin tumors,
involving lower extremities and/or
lymph nodes
Disseminated mucocutaneous and
visceral involvement
Localized mucocutaneous or
disseminated KS, with possible
visceral lesions
Behavior
Indolent
Progressive; lymphadenopathic
form is aggressive
Aggressive; lesions may regress or
flare with initiation of
antiretroviral therapy
Variable; may regress after
immunosuppression is
discontinued

Africa, however, has begun to show an equal incidence of KS HISTOPATHOLOGY

in men and women. A second non-KS hematologic
malignancy is often present in patients with classic KS.
African KS follows a more aggressive course than classic KS,
especially the lymphadenopathic form that affects young
individuals. In HIV-infected persons, KS is an AIDS-defining
illness. AIDS-associated KS (AIDS-KS) usually, but not
exclusively, arises in HIV-positive patients with low CD4 T-
cell counts.10 AIDS-KS is a much more aggressive disease
that typically manifests with disseminated lesions (Figure 1)
and visceral involvement. This may be attributed to the fact
that HIV infection augments HHV8 replication. Kaposi
sarcoma exhibits a less aggressive presentation in patients
already receiving highly active antiretroviral therapy
(HAART). Exacerbation (also called KS flare) can occur after
therapy (eg, corticosteroids, rituximab) or subsequent to the
immune reconstitution inflammatory syndrome that may
occur when initiating HAART in HIV-infected persons.11
Iatrogenic KS is associated with immunosuppression due to
drugs or after transplantation. Kaposi sarcoma occurs mainly
in renal transplant recipients, and infrequently after other
solid organ or bone marrow transplants. Posttransplant KS
may result from reactivation of latent HHV8 infection in
recipients or from tumor cells contributed from organ donors.
Transplant-associated KS has a protracted, but aggressive
course. Fortunately, in transplant recipients, KS lesions may
regress after discontinuation of immunosuppressive therapy.
In all of the aforementioned clinical settings, KS lesions
evolve from early (patch stage) macules into plaques (plaque
stage) that may subsequently develop into larger nodules
(tumor stage). These tumors may ulcerate, cause marked
lymphedema, present as exophytic growths (eg, cutaneous
horns), or invade subjacent tissues (eg, underlying bone).
Different stages can coexist in the same individual at the same
time. Similar stages of KS growth apply to both cutaneous and
mucosal lesions. While KS commonly presents at mucocuta-
neous sites, tumors may involve lymph nodes as well as
visceral organs, most notably the respiratory and gastrointes-
tinal tracts. Extracutaneous KS has also been described in
unusual anatomic locations including the musculoskeletal
system, nervous system, heart, wounds, within pemphigus
lesions, and in blood clots.12 A 2-tiered staging system exists
for AIDS-related KS, according to the AIDS Clinical Trials
Group (ACTG)13: T0 is used for KS confined to the skin and
lymph nodes with minimal oral involvement, and T1 refers to
KS with ulceration or associated edema, nodular oral KS, or
KS involvement of any other visceral organ. A different
staging system is available for classic KS to facilitate
therapeutic decision making.
290 Arch Pathol Lab MedVol 137, February 2013
The histopathology of KS is essentially identical in the
different epidemiologic KS types. Nevertheless, some
studies have documented minor histopathologic differences
between AIDS-KS and non-HIVassociated KS cases,
namely, that mitoses and cellular anaplasia are more
common in HIV-negative patients, whereas AIDS-KS
lesions tend to display more extensive dissecting vessels.
Early patch-stage KS is characterized by abnormal vessels
lined by thin endothelial cells dissecting the dermis.
Ramifying proliferating vessels often surround larger ectatic
vessels and skin adnexa, producing the so-called promon-
tory sign. This sign is not pathognomonic for KS, as it has
also been described in other vascular lesions including
benign vascular tumors and angiosarcoma. Sparse chronic
inflammatory cells, extravasated red blood cells, and
hemosiderin-laden macrophages are frequently present in
patch KS lesions. These early histologic changes may be
inconspicuous, and for that reason can be easily missed on
biopsy.14
Plaque-stage KS lesions are characterized by a prolifera-
tion of both spindle cells and vessels, which in the skin
involve most of the dermis, and sometimes even the
subcutis. Well-developed KS tumors consist of several
fascicles of these spindle-shaped tumor cells (Figure 2),
often admixed with a variable chronic inflammatory infiltrate
composed of lymphocytes, plasma cells, and dendritic cells.
Kaposi sarcoma lesions also contain several hemosiderin-
laden macrophages. This is not surprising as iron appears to
be important in the pathogenesis of KS. Iron staining may
help distinguish KS from similar-appearing interstitial
granuloma annulare lesions that lack iron. In cross section,
KS nodules display a sievelike appearance caused by the
transection of spindle cells with intervening slitlike spaces.
Fine-needle aspiration specimens show cohesive clusters of
these spindle cells, typically present in a bloody background
(Figure 3). Eosinophilic and periodic acid-Schiff (PAS)
positive hyaline globules are a common finding in advanced
KS lesions. These globules may be located within lesional
cells or extracellularly.15 Electron microscopy may show
occasional Weibel-Palade bodies within lesional cells, as
well as intracellular fragmented erythrocytes that are
believed to correspond to the hyaline globules seen by light
microscopy. Typical KS lesions are devoid of marked cellular
pleomorphism, necrosis, or a significant number of mitotic
figures. In rare instances, AIDS-KS lesions may harbor
concomitant pathologic findings, usually an opportunistic
Kaposi SarcomaRadu & Pantanowitz

Figure 1. Human immunodeficiency viruspositive patient showing
disseminated AIDS-associated cutaneous Kaposi sarcoma plaques on
his body (image courtesy of Bruce J. Dezube, MD).
Figure 2. Kaposi sarcoma tumor showing fascicles of spindled cells with
extravasated red blood cells and scattered chronic inflammatory cells
(hematoxylin-eosin, original magnification 3200).
pathogen (eg, cryptococcosis, mycobacterial granulomas, or
molluscum contagiosum).16
In patients with HIV infection, HAART may lead to partial
or complete regression (reduced size, flattening, and color
Arch Pathol Lab MedVol 137, February 2013
change from violaceous to brown) of KS lesions. Partially
regressed lesions have some residual spindled cells.
However, with complete regression, spindle cells are absent.
Completely regressed lesions have an increase in dermal
capillary density around native dermal vessels and append-
ages, an increased perivascular infiltrate containing many
plasma cells, and numerous hemosiderin-laden dermal
macrophages.17 Regressed lesions may also become scle-
rotic, especially those that are subjected to intralesional
chemotherapy injection.
HISTOLOGIC VARIANTS
Several histologic variants of KS have been described
(Figure 4, A through D).18,19 Their clinical significance
remains largely unknown. The spectrum of KS has been
expanded to include pre-KS lesions, also referred to as an
in situ form of KS.20 These pre-KS lesions are character-
ized by groups of abnormal capillary-like vessels admixed
with an inflammatory infiltrate, similar to patch-stage KS
lesions. They are associated with lymphangiogenesis arising
in the setting of chronic lymphedema. On the other end of
the spectrum is anaplastic KS, sometimes referred to as
pleomorphic KS. Anaplastic KS is an infiltrative, solid
proliferation of spindle cells without vascular spaces, seen
mainly in AIDS involving acral sites.21 This aggressive
variant displays a greater degree of cellular and nuclear
atypia, high mitotic index (eg, 5 to 20 mitoses per 10 high-
power fields), and occasional necrosis. There are also several
lymphedematous variants, best classified according to their
association with ectatic lymphatics (lymphangioma-like KS
and lymphangiectatic KS) or due to the accumulation of
dermal and/or intradermal edema (bullous KS).22 Lymph-
angioma-like KS (or lympangiomatous) KS is characterized
by many ectatic, interanastomosing vascular channels
usually devoid of erythrocytes. Some of these channels
may closely abut the overlying epidermis, analogous to
lymphangioma circumscriptum. This differs from the
telangiectatic variant of KS in which KS lesions contain
large, very congested, ectatic vascular spaces. With ecchy-
motic KS, on the other hand, the intradermal KS
proliferation is accompanied by extensive red blood cell
extravasation (purpura effect), often so marked that it
almost obscures the underlying histologic features of KS.
Glomeruloid KS is used to describe KS lesions, or areas
within KS lesions, that contain relatively circumscribed,
congested glomeruloid vascular structures. Intravascular KS
is reserved for those rare cases that have an exclusively
intravascular solid spindle cell proliferation.23
Hyperkeratotic (verrucous) KS is related to severe KS-
associated lymphedema that causes verrucous epidermal
acanthosis, hyperkeratosis, and fibrosis overlying a deep KS
lesion. Lymphedematous AIDS-KS may also be associated
with exophytic dermal fibroma-like nodules. In keloidal KS,
there is marked expansion of the dermis by dense,
hyalinized collagen that resembles a keloid.24 Because the
abundant collagen obscures the KS spindled cells, this
variant may be mistaken for scar tissue. Micronodular KS
refers to nodular KS lesions characterized histologically by a
small, unencapsulated, circumscribed spindle cell prolifera-
tion in the reticular dermis. These small KS tumors may
sometimes be entirely removed in a skin punch biopsy.
Superficially located KS lesions that protrude from the skin
and elicit a peripheral epidermal collarette may resemble a
pyogenic granuloma (PG); such lesions have accordingly
Kaposi SarcomaRadu & Pantanowitz 291
Figure 3. Cytology specimen of a Kaposi sarcoma lesion showing a cohesive group of spindled tumor cells present in a bloody background
(hematoxylin-eosin, original magnification 3200).
Figure 4. Different Kaposi sarcoma (KS) variants. A, Anaplastic KS. B, Telangiectatic KS. C, Glomeruloid KS. D, Keloidal KS. Images courtesy of
Wayne Grayson, MBBCh, PhD, FCPath (hematoxylin-eosin, original magnifications 3400 [A and C] and 3200 [B]; Masson trichrome, original
magnification 3200 [D]).
Figure 5. Kaposi sarcoma spindle tumor cells showing nuclear LNA-1 (latency-associated nuclear antigen) staining (original magnification 3600).

been referred to as pyogenic granulomalike KS. In KS with
myoid nodules, nodular KS is associated with intratumoral
myoid nodules, similar to those that may be seen in some
dermatofibrosarcoma protuberans lesions. Pigmented KS is
292 Arch Pathol Lab MedVol 137, February 2013
another rare variant attributed to the presence of increased
numbers of melanin-laden dendritic cells intimately ad-
mixed with KS spindle tumor cells, often seen in paucivas-
cular anaplastic KS.25 Kaposi sarcoma with sarcoidlike
Kaposi SarcomaRadu & Pantanowitz
granulomas has also been observed, which appears to be
related to mycobacterial infection in AIDS-KS lesions.26
IMMUNOHISTOCHEMISTRY
More than 100 different primary antibodies have been
evaluated in KS with immunohistochemistry.27 Kaposi
sarcoma lesional cells stain positively with the endothelial
markers factor VIIIrelated antigen, CD31 (PECAM-1), and
CD34. CD34 tends to show stronger expression than CD31
in advanced-stage lesions of KS. Kaposi sarcoma spindle
cells also express several lymphatic specific markers such as
D2-40 (which binds to the podoplanin antigen), LYVE-1 (a
homologue of the CD44 glycoprotein receptor for hyalur-
onan), VEGFR-3 (the receptor for vascular endothelial
growth factor C), and Prox-1. Bcl-2 also shows positivity
in KS, related to the tumors mechanisms of resisting
apoptosis. The identification and localization of HHV8
within KS lesional cells by using LNA-1 is the most
diagnostically helpful immunostaining technique available
to differentiate KS from its mimics. LNA-1 immunoreac-
tivity in KS cells appears as stippled nuclear staining (Figure
5). However, HHV8 is not entirely limited to KS and has
been detected in some angiosarcomas, hemangiomas, and
dermatofibromas.28 LNA-1 immunohistochemistry is fa-
vored over polymerase chain reaction detection of HHV8 in
the evaluation of problematic vascular proliferations because
contaminating mononuclear inflammatory cells may also
harbor this herpesvirus, especially in HIV-positive patients.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis of KS and its variants is broad.
Clinical history, such as HIV infection or status post
transplant, may strongly support the diagnosis of KS. Even
in these clinical settings, patch-stage cutaneous KS lesions
may need to be differentiated from targetoid hemosiderotic
hemangioma, fibrous histiocytoma, and interstitial granu-
loma annulare. The histologic differential diagnosis of
plaque-stage KS includes tufted angioma, targetoid hemo-
siderotic hemangioma, microvenular hemangioma, and
acroangiodermatitis (pseudo-Kaposi sarcoma). Lesions different epidemiologic forms of KS. Optimal control of HIV
that may potentially be confused with nodular KS include
bacillary angiomatosis, other vascular tumors (eg, spindle
cell hemangioma and Kaposiform hemangioendothelioma),
fibrohistiocytic tumors (eg, cellular, angiomatoid, and
atypical variants of fibrous histiocytoma, and dermatofibro-
sarcoma protuberans), resolving dermal fasciitis, spindle cell
melanoma, and several other spindle cell mesenchymal
neoplasms (eg, cutaneous leiomyosarcoma). Advanced and
more aggressive forms of KS need to be differentiated from
angiosarcoma.
Paucivascular anaplastic KS needs to be distinguished
from other high-grade sarcomas (eg, leiomyosarcoma,
spindle cell rhabdomyosarcoma, malignant peripheral nerve
sheath tumor, fibrosarcoma), amelanotic spindle cell mel-
anoma, and spindle cell carcinoma. Pigmented anaplastic
KS may mimic melanoma, especially when present on acral
sites. Lymphangioma-like KS may resemble other benign
lymphatic tumors such as lymphangioma circumscriptum or
a lymphangioendothelioma/acquired progressive lymphan-
gioma. The architectural arrangement of telangiectatic KS
may mimic a sinusoidal hemangioma, an uncommon
acquired variant of cavernous hemangioma. Keloidal KS
may be mistaken for a true keloid scar, especially if there has
been a previous nearby biopsy. Pyogenic granulomalike KS
Arch Pathol Lab MedVol 137, February 2013
may be misdiagnosed as a true PG (lobular capillary
hemangioma), especially if the surface is traumatized and
ulcerated. The differential diagnosis of intravascular KS
includes intravascular papillary endothelial hyperplasia,
intravenous PG, intravascular fasciitis, papillary intralym-
phatic angioendothelioma (Dabska tumor), and intravascu-
lar myopericytoma. Finally, without any prior clinical
knowledge that KS lesions have responded to therapy,
regressed KS lesions may be misdiagnosed clinically and
histologically as pigmented purpuric dermatitis. For most of
the aforementioned vascular entities, immunohistochemis-
try using vascular markers (CD31 or CD34) and/or
lymphatic markers (D2-40) is not as specific as LNA-1 in
diagnosing KS.
THERAPY AND PROGNOSIS
Treatment goals of KS include symptom palliation,
prevention of KS progression, improvement of cosmesis,
and abatement of associated edema, organ compromise,
and psychologic stress.29 At present there is no cure for KS.
Surgical excision is restricted for cosmetically disturbing KS
lesions, to alleviate discomfort, or to control local tumor
growth. Other local therapies used to manage bulky lesions
or for cosmesis include external beam radiation, laser
therapy, cryotherapy, photodynamic therapy, topical alitre-
tinoin gel, and intralesional vinblastine. Indications for
systemic chemotherapy include widespread skin involve-
ment (.25 lesions), extensive oral KS, marked symptomatic
edema, rapidly progressive disease, symptomatic visceral
KS, and KS flare. Currently, liposomal anthracyclines and
taxanes are the backbone of systemic cytotoxic therapy
against KS. Several novel therapies have been tried with
some success including interferon a, thalidomide, antiher-
pes therapy, imatinib and matrix metalloproteinase inhib-
itors (eg, COL-3). While HHV8 is susceptible to antiviral
medications (eg, ganciclovir) in the lytic phase, unfortu-
nately most cells in KS lesions harbor HHV8 in its latent
phase.
Apart from HAART, treatment options are similar for the
infection, using antiretroviral therapy, is a key component in
the treatment of AIDS-KS. HAART has greatly decreased
the incidence of AIDS-KS. Before the advent of HAART, the
ACTG staging system for AIDS-KS correlated well with
survival. Since the introduction of HAART, factors sugges-
tive of an unfavorable prognosis include age 50 years or
older, positive HHV8 DNA finding in plasma at the time of
diagnosis, advanced KS with systemic disease (especially
pulmonary lesions), and a concomitant AIDS-associated
illness. For iatrogenic KS, adjustment of immunosuppres-
sive therapy may be necessary, as well as the introduction of
sirolimus (rapamycin) for patients with posttransplant KS.
The discovery of HHV8 and related data about the
pathogenesis of KS have resulted in the identification of
multiple novel therapeutic targets (eg, bevacizumab target-
ing possible inhibition of vascular endothelial growth
factor), many of which are being investigated in ongoing
clinical trials.30
CONCLUSIONS
Kaposi sarcoma is a low-grade vascular tumor of endo-
thelial origin that is associated with HHV8 infection. The
extent and aggressiveness of KS tumors depends on the
epidemiology (classic, African, AIDS-associated, and iatro-
Kaposi SarcomaRadu & Pantanowitz 293
genic KS) and host immunity. The histopathology in these
different KS forms is essentially identical. Pathologists need
to be aware that there are several newer histologic variants
and clinical manifestations, including KS regression and
exacerbation. LNA-1 (HHV8) is the most specific immuno-
histochemical marker available to help confirm the diagnosis
and distinguish KS from its mimics. In some regions of the
world, particularly sub-Saharan Africa, AIDS-KS remains the
most common HIV-associated malignancy encountered and
is therefore the leading cancer diagnosed. Fortunately, the
expanding knowledge of KS biology is providing increasing
opportunities for rational targeted therapies.
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