KAPOSI’S KAPOSI’S
SARCOMASARCOMA
By john masinaaBy john masinaa
Out LineOut Line
DefinitionDefinition
PathophysiologyPathophysiology
Clinical CharacteristicsClinical Characteristics
Diagnosis and Differential DiagnosisDiagnosis and Differential Diagnosis
TreatmentTreatment
PrognosisPrognosis
Def: Def:
It is a cancer arising from capillary endothelial It is a cancer arising from capillary endothelial
cells.cells.
-caused by a virus called -caused by a virus called Human Herpes Virus Human Herpes Virus
type 8 (HHV8)type 8 (HHV8)
-Most prominent AIDS defining Cancer-Most prominent AIDS defining Cancer
The tumours appear as red or purple patches The tumours appear as red or purple patches
on the skin , mouth, lung, liver or gastro-on the skin , mouth, lung, liver or gastro-
intestinal tractintestinal tract
TYPESTYPES
1.1.Classic Kaposi’s SarcomaClassic Kaposi’s Sarcoma
2.2.African Kaposi’s SarcomaAfrican Kaposi’s Sarcoma
3.3.Immunosuppressive-treatment-Immunosuppressive-treatment-
related Kaposi’s Sarcomarelated Kaposi’s Sarcoma
4.4.AIDS-related Kaposi’s Sarcoma- AIDS-related Kaposi’s Sarcoma-
occurs in people with late stage occurs in people with late stage
HIV/AIDS. It is a rapidly progressing HIV/AIDS. It is a rapidly progressing
tumour and very aggressivetumour and very aggressive
PATHOPHYSIOLOGYPATHOPHYSIOLOGY
Kaposi’s Sarcoma lesionsKaposi’s Sarcoma lesions contain contain
tumour cells with a characteristic tumour cells with a characteristic
abnormal elongated shape, called abnormal elongated shape, called
spindle cellsspindle cells. The tumuor is highly . The tumuor is highly
vascular, containing abnormally vascular, containing abnormally
dense and irregular vessels which dense and irregular vessels which
leak RBCs into surrounding tissue leak RBCs into surrounding tissue
and give the tumour its dark and give the tumour its dark
colourcolour..
CLINICAL FEATURESCLINICAL FEATURES
SYMPTOMSSYMPTOMS
Swollen glandsSwollen glands
Swollen feetSwollen feet
Purple (dark) Purple (dark)
papules on the papules on the
skin, mucosa and skin, mucosa and
viscera in late viscera in late
diseasedisease
SIGNSSIGNS
LymphadenopathyLymphadenopathy
Woody swollen feetWoody swollen feet
DIAGNOSISDIAGNOSIS
From History and Physical From History and Physical
ExaminationExamination
DIFFERANTIAL DIAGNOSISDIFFERANTIAL DIAGNOSIS
1.1.Lymphoma Lymphoma
2.2.HemangiomaHemangioma
3.3.Erythema nodosumErythema nodosum
Definitive Diagnosis: Biopsy and Definitive Diagnosis: Biopsy and
MicroscopyMicroscopy
Detection of LANA in tumour cells Detection of LANA in tumour cells
confirms the diagnosisconfirms the diagnosis
DIAGNOSISDIAGNOSIS
OTHER INVESTIGATIONSOTHER INVESTIGATIONS
Full Blood CountFull Blood Count
Chest X-rayChest X-ray
Retroviral serology- HIV 1, HIV 2; CD4+Retroviral serology- HIV 1, HIV 2; CD4+
TREATMENTTREATMENT
KS is not curable, in the usual sense of the KS is not curable, in the usual sense of the
word.word.
AIM:AIM: to ease symptoms, shrink the tumour to ease symptoms, shrink the tumour
and prevent the disease from getting worse and prevent the disease from getting worse
i.e. Palliativei.e. Palliative
The type of treatment depends on the type of The type of treatment depends on the type of
KS, the size of the tumour, the extent of KS, the size of the tumour, the extent of
internal organ involvement and the person’s internal organ involvement and the person’s
CD4 count and general medical conditionCD4 count and general medical condition
In >40% of pts with AIDS-associated KS, the In >40% of pts with AIDS-associated KS, the
lesions will shrink upon first starting HAART.lesions will shrink upon first starting HAART.
ChemotherapyChemotherapy
Local or intralesional chemotherapy: Local or intralesional chemotherapy:
esp. in the oral cavity- Vinblastine is given esp. in the oral cavity- Vinblastine is given
every 3 weeks for a total of 2-3 doses.every 3 weeks for a total of 2-3 doses.
Systemic ChemotherapySystemic Chemotherapy
The following combinations can be The following combinations can be
givengiven
Vinblastine + VincristineVinblastine + Vincristine
Bleomycin + Vinblastine or VincristineBleomycin + Vinblastine or Vincristine
Bleomycin + Doxorubicin + Vincristine Bleomycin + Doxorubicin + Vincristine
or Vinblastineor Vinblastine
--Plus ARVs--Plus ARVs
The disease is extensive and develops The disease is extensive and develops
fast therefore all patients should fast therefore all patients should
receive combination therapyreceive combination therapy
If the general condition of the pt is very If the general condition of the pt is very
poor e.g. very malnourished, it is poor e.g. very malnourished, it is
advisable to start with monotherapy- advisable to start with monotherapy-
Vincristine along with nutrition Vincristine along with nutrition
supplement (HEPS)supplement (HEPS)
TB and Pregnancy are absolute TB and Pregnancy are absolute
contraindicationscontraindications
STANDARD TREATMENT GUIDELINES 2004- STANDARD TREATMENT GUIDELINES 2004-
CBoHCBoH
STAGESSTAGES
Stage 1: lymph node involvement only; Stage 1: lymph node involvement only;
cutaneous, locally indolent.cutaneous, locally indolent.
Stage 2: Locally aggressive, with or without Stage 2: Locally aggressive, with or without
involvement of regional lymph nodes.involvement of regional lymph nodes.
Stage 3: Generalized aggressive, with or Stage 3: Generalized aggressive, with or
without lymph node involvementwithout lymph node involvement
Stage 4: Visceral disease e.g. lung Stage 4: Visceral disease e.g. lung
involvementinvolvement
HHV 8- discovered in 1994 may actually HHV 8- discovered in 1994 may actually
be the causal agentbe the causal agent
Recent studies show that men with HHV8 Recent studies show that men with HHV8
are nearly 12 times more likely to be are nearly 12 times more likely to be
diagnosed with KS than those without diagnosed with KS than those without
HHV 8HHV 8
DRUGSDRUGS
Interferon Interferon alphaalpha
Liposomal anthracyclines- drugs Liposomal anthracyclines- drugs
enclosed in tiny fat globules that are enclosed in tiny fat globules that are
better taken up the tumour cells: better taken up the tumour cells:
-Doxorubicin; Daunorubicin-Doxorubicin; Daunorubicin
PaclitaxelPaclitaxel
RECENT ADVANCEMENTSRECENT ADVANCEMENTS
Angiogenesis Inhibitors: block Angiogenesis Inhibitors: block
development of blood vessels within development of blood vessels within
tumourstumours
The recent discovery of KSHV and HHV8 The recent discovery of KSHV and HHV8
also offers a new target for KS drugs also offers a new target for KS drugs
and Biological Therapyand Biological Therapy