Kaposi’s Sarcoma
Dr. O Ngalamika
1
 First described by Moritz Kaposi in 1872
 Most common cutaneous malignancy in HIV
disease
 Various visceral organs can also be affected
 It has several subtypes which have different
presentations, epidemiology, and prognoses
2
Etiopathogenesis
 Formed by abnormal proliferation of vascular
endothelial cells
 Infection with HHV-8 has been associated
with the development of KS
 HHV-8 is found in KS lesional tissue
irrespective of clinical type
 Transmission of HHV-8: mainly through
saliva. Organ transplantation, blood
transfusion
3
 HHV-8 is known to encode products that lead
to growth dysregulation or evasion of immune
surveillance
4
Clinical Features
 Characterized by brown, pink, red or
violaceous macules/patches,
papules/plaques, nodules
 Lesions may vary depending on the clinical
variant
 Mucous membrane, cutaneous and visceral
involvement is common (lymph nodes, GIT,
and lungs)
5
6
7
KS Subtypes
8
1. Classic KS
 Indolent disease
 Seen chiefly in middle-aged men of southern
and eastern European origin
 Early lesions appear most commonly on the
toes or soles as reddish, violaceous, or
bluish-black macules and patches that
spread and coalesce to form nodules or
plaques
9
 Brawny edema of the affected limb may be
present
 Macules or nodules may appear, usually
much later, on the arms and hands, and
rarely may extend to the face, ears, trunk,
genitalia, or oral mucosa
 Classic KS has a slowly progressive course
 In the early stages of disease, lesions may
undergo spontaneous resolution
10
2. African cutaneous KS
 Endemic in tropical Africa
 Mostly seen in men between the ages of 20
and 50
 It has a locally aggressive but systemically
indolent course
 Characterized by nodular, infiltrating,
vascular masses on the extremities
11
3. African lymphadenopathic KS
 An aggressive disease of young patients,
mainly children under age 10
 Lymph node involvement with or without skin
lesions
 Has an aggressive course, often terminating
fatally within 2 years of onset
12
4. AIDS-associated KS
 KS in patients immunosuppressed by AIDS
 Cutaneous lesions begin as one or several
red to purple-red macules, rapidly
progressing to papules, nodules, and plaques
 There is a predilection for the head, neck,
trunk, and mucous membranes
 A fulminant, progressive course with nodal
and systemic involvement is expected
13
 It may be the presenting manifestation of HIV
infection (HIV stage 4)
14
5. Immunosuppression-associated KS
 Occurs in patients on immunosuppressive
therapy
 Clinical features may be similar to that of
classic KS; however, site of presentation is
more variable
 Removal of the immunosuppression may
result in regression of the KS
15
16
17
Internal involvement
 Classic KS: GIT is most frequently involved.
Lungs, heart, liver, conjunctiva, adrenal
glands, abdominal lymph nodes, and bones
may also be affected.
 African cutaneous KS: frequently
accompanied by massive leg edema and
frequent bone involvement
 African lymphadenopathapic KS:
Reported among Bantu children who develop
massive lymphnode involvement, preceding
appearance of skin lesions
18
- The children also develop lesions on the
eyelids and conjunctiva
- Eye involvement is often associated with
swelling of the lacrimal, parotid, and
submandibular glands
 AIDS-associated KS: Lungs (37%),
gastrointestinal tract (50%), and lymph nodes
(50%)
19
Epidemiology
 KS is worldwide in distribution
 In africa, it occurs largely in the south of the sahara
 Prevalence of AIDS-related KS has decreased due
to widespread availability of HAART
 KS associated with other forms of
immunosuppression include those with iatrogenic
suppression from oral prednisone or other chronic
immunosuppressive therapies, as may be given to
transplant patients
 It affects more men than women. Ratio ~ 2:1
20
Differential diagnosis
 Bacillary angiomatosis
 Pyogenic granuloma
 Leukemia cutis
 Cellulitis
 Severe stasis dermatitis
 Small-vessel vasculitis
21
Work-up
 Baseline investigations
 Skin biopsy
 Immunohistochemistry
 HIV test
 HHV-8 PCR
 CXR/CT-Scan
 Endoscopy
22
Histopathology
 Prominent spindle cells
 Prominent slitlike vascular spaces
 Extravasated red blood cells
23
Treatment
 Topical retinoid
 Surgical excision
 Radiation
 Laser
 ART for epidemic KS
24
 Chemotherapy:
- liposomal doxorubicin, liposomal
daunorubicin
- Vincristine, Bleomycin, Doxorubicin: 1st
line at
UTH/CDH
- Paclitaxel monotherapy: 2nd
line agent
25
Course
 Classic KS: Progresses slowly, rare lymph node or
visceral involvement. Death occurs years later from
unrelated causes
 African cutaneous KS: aggressive, early nodal
involvement, death from KS expected within 1-2
years
 AIDS-related KS: although widespread, most
patients die of intercurrent infection
 Immunosuppression related KS: removal of
immunosuppression may result in resolution of the
KS without therapy
26
End
27

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03. Kaposi’s Sarcoma.ppt dermatological condition

  • 2.  First described by Moritz Kaposi in 1872  Most common cutaneous malignancy in HIV disease  Various visceral organs can also be affected  It has several subtypes which have different presentations, epidemiology, and prognoses 2
  • 3. Etiopathogenesis  Formed by abnormal proliferation of vascular endothelial cells  Infection with HHV-8 has been associated with the development of KS  HHV-8 is found in KS lesional tissue irrespective of clinical type  Transmission of HHV-8: mainly through saliva. Organ transplantation, blood transfusion 3
  • 4.  HHV-8 is known to encode products that lead to growth dysregulation or evasion of immune surveillance 4
  • 5. Clinical Features  Characterized by brown, pink, red or violaceous macules/patches, papules/plaques, nodules  Lesions may vary depending on the clinical variant  Mucous membrane, cutaneous and visceral involvement is common (lymph nodes, GIT, and lungs) 5
  • 6. 6
  • 7. 7
  • 9. 1. Classic KS  Indolent disease  Seen chiefly in middle-aged men of southern and eastern European origin  Early lesions appear most commonly on the toes or soles as reddish, violaceous, or bluish-black macules and patches that spread and coalesce to form nodules or plaques 9
  • 10.  Brawny edema of the affected limb may be present  Macules or nodules may appear, usually much later, on the arms and hands, and rarely may extend to the face, ears, trunk, genitalia, or oral mucosa  Classic KS has a slowly progressive course  In the early stages of disease, lesions may undergo spontaneous resolution 10
  • 11. 2. African cutaneous KS  Endemic in tropical Africa  Mostly seen in men between the ages of 20 and 50  It has a locally aggressive but systemically indolent course  Characterized by nodular, infiltrating, vascular masses on the extremities 11
  • 12. 3. African lymphadenopathic KS  An aggressive disease of young patients, mainly children under age 10  Lymph node involvement with or without skin lesions  Has an aggressive course, often terminating fatally within 2 years of onset 12
  • 13. 4. AIDS-associated KS  KS in patients immunosuppressed by AIDS  Cutaneous lesions begin as one or several red to purple-red macules, rapidly progressing to papules, nodules, and plaques  There is a predilection for the head, neck, trunk, and mucous membranes  A fulminant, progressive course with nodal and systemic involvement is expected 13
  • 14.  It may be the presenting manifestation of HIV infection (HIV stage 4) 14
  • 15. 5. Immunosuppression-associated KS  Occurs in patients on immunosuppressive therapy  Clinical features may be similar to that of classic KS; however, site of presentation is more variable  Removal of the immunosuppression may result in regression of the KS 15
  • 16. 16
  • 17. 17
  • 18. Internal involvement  Classic KS: GIT is most frequently involved. Lungs, heart, liver, conjunctiva, adrenal glands, abdominal lymph nodes, and bones may also be affected.  African cutaneous KS: frequently accompanied by massive leg edema and frequent bone involvement  African lymphadenopathapic KS: Reported among Bantu children who develop massive lymphnode involvement, preceding appearance of skin lesions 18
  • 19. - The children also develop lesions on the eyelids and conjunctiva - Eye involvement is often associated with swelling of the lacrimal, parotid, and submandibular glands  AIDS-associated KS: Lungs (37%), gastrointestinal tract (50%), and lymph nodes (50%) 19
  • 20. Epidemiology  KS is worldwide in distribution  In africa, it occurs largely in the south of the sahara  Prevalence of AIDS-related KS has decreased due to widespread availability of HAART  KS associated with other forms of immunosuppression include those with iatrogenic suppression from oral prednisone or other chronic immunosuppressive therapies, as may be given to transplant patients  It affects more men than women. Ratio ~ 2:1 20
  • 21. Differential diagnosis  Bacillary angiomatosis  Pyogenic granuloma  Leukemia cutis  Cellulitis  Severe stasis dermatitis  Small-vessel vasculitis 21
  • 22. Work-up  Baseline investigations  Skin biopsy  Immunohistochemistry  HIV test  HHV-8 PCR  CXR/CT-Scan  Endoscopy 22
  • 23. Histopathology  Prominent spindle cells  Prominent slitlike vascular spaces  Extravasated red blood cells 23
  • 24. Treatment  Topical retinoid  Surgical excision  Radiation  Laser  ART for epidemic KS 24
  • 25.  Chemotherapy: - liposomal doxorubicin, liposomal daunorubicin - Vincristine, Bleomycin, Doxorubicin: 1st line at UTH/CDH - Paclitaxel monotherapy: 2nd line agent 25
  • 26. Course  Classic KS: Progresses slowly, rare lymph node or visceral involvement. Death occurs years later from unrelated causes  African cutaneous KS: aggressive, early nodal involvement, death from KS expected within 1-2 years  AIDS-related KS: although widespread, most patients die of intercurrent infection  Immunosuppression related KS: removal of immunosuppression may result in resolution of the KS without therapy 26