KS is a AIDS defining illness with a course ranging from indolent, with only skin manifestations to fulminant, with extensive visceral involvement.

1. Kaposi’s Sarcoma

2. Kaposi sarcoma was
first described as
‘idiopathic multiple
pigmented sarcoma of
the skin’ by the
Hungarian dermatologist
Moritz Kaposi in 1872.

3.  From that time until the HIV disease epidemic
identified with AIDS, KS remained a rare tumor.
 It became more widely known as one of the
AIDS-defining illnesses in the 1980s.

4. Causative agent
 KSHV was discovered as a causative agent in
1994.
 Kaposi sarcoma (KS) is a multifocal, endothelial
proliferation caused by human herpesvirus 8
(HHV‐8)
 The disease is multifocal, with a course ranging
from indolent, with only skin manifestations to
fulminant, with extensive visceral involvement.

5. • Although HHV‐8 is considered the causative
agent, it is insufficient to cause KS alone.
• Multiple co‐factors are required, the most
powerful of which is HIV co‐infection, which
elevates the risk up to 20,000‐fold.

6.  There are four distinct clinicopathological
subtypes:
 ➤ Classic (Sporadic KS)
 ➤ Endemic (sub-Saharan Africa)
 ➤ Iatrogenic (transplant recipients)
 ➤ AIDS associated (Epidemic KS)

7.  KS in MSM without HIV infection is increasingly
being recognized as a possible distinct fifth form
of KS

8. • Incidence varies dramatically from less than
1 per 100,000 in western Europe and North
America to over 22 per 100,000 in central
Africa where the disease occurs in its
endemic form and HIV infection is rampant.

11.  Classic KS occurs mostly in elderly men of
Mediterranean or Jewish ancestry
 10-15 times more common in men than women.
 Chronic lymphedema is a frequent association.
Classic KS

12.  It typically shows an indolent, protracted clinical
course and primarily affects skin on the legs,
though lymph nodes & visceral mucosa may also
be involved.
 Slow progression with good prognosis.
 Occasionally an aggressive type of Kaposi’s
sarcoma occurred in young adults or children
with early lymph node involvement.

13. African / Endemic KS
 Presents in young adults median age 37.
 Males > Females
 Accounts for up to 9% of all reported cancers in
equatorial Africa.
 Poor prognosis

14. • In South Africa, it has been estimated that
Kaposi’s sarcoma is ten times more common in
blacks than whites.
• In Africans, the disease can run a similar course
as in Classic KS, but a higher proportion of
young people are affected, with a more
aggressive disease manifestation.

15. • Most cases in children, with or without HIV
infection, are of the lymphadenopathic type and
are rapidly fatal due to visceral dissemination.

16. 4 clinical patterns
 Nodular type: benign course & resembles
classic KS.
 Florid / Vegetating type: More aggressive,
extend deeply into the subcutis, muscle & bone.
 Infiltrative type: More aggressive. Has
mucocutaneous & visceral involvement.
 Lymphadenopathic type: Seen in children
(male:female = 3:1) & young adults. Viscera &
massive LN involvement. Early death.

18.  Fungating lesions
seen in Florid /
Vegetating type of
KS

19. Iatrogenic KS
 Most common in solid organ transplant
recipients.
 Chronic use of immunosupressive drugs.
 Cyclosporine is associated with a higher
incidence and more rapid onset of disease.
 Resolves on cessation of immunosupression.

20.  Incidence of KS is reported to currently be
~200- fold higher in recipients of solid-organ
transplants (that is, in iatrogenic KS) than in the
general population
 No sex predilection.

21. HIV associated KS
 Risk 20,000 times more in AIDS patient
compared to general population.
 Common in homosexual men.
 Visceral involvement may be present without any
skin lesions

22. Rapid progression, atypical distribution affecting the
trunk & extensive systemic involvement in HIV.

23. Disseminated AIDS-related KS in a 36-year-old
man. Nodular violaceous involvement of the tongue
base & soft and hard palates.

24.  Multifocal involvement of respiratory system can
be asymptomatic or lead to dyspnea, cough,
hemoptysis and/or chest pain.
 GI complications include ulceration, massive
haemorrhage, perforation and ileus. Small
intestine is the most common location.

25.  KS of bowel and/or lungs is responsible for
numerous deaths.
 HIV-associated KS is an aggressive tumor that
results in a median survival time of 18 months
without treatment.

26. Diagnosis
• Dermoscopy - Dermoscopic examination
reveals
➤ bluish-reddish coloration
➤ areas showing various colours of the
rainbow spectrum
➤ scaly surface
• Skin biopsy – Vascular channels lined by
atypical endothelial cells. Extravasated
erythrocytes with hemosiderin deposition.

27. • Immunohistochemistry – LANA-1, CD31,
CD34, D2-40 positivity
• Imaging – Abdominal ultrasound, CXR,
HRCT, (extent of visceral involvement)

28. Dermoscopic findings (non-polarized light)
 White lines & clods
 Purplish-pinkish colouration
 Surface scale
 Rainbow pattern

29. purple-white colour, polychromatic colour change,
surface scale, haemorrhagic clot

30. Dermoscopic findings (polarized light)
 purplish-pinkish coloration
 rainbow pattern
 scaly surface
 small brown globules

31. bluish-reddish colouration (a, b), scaly surface (c)

32. The ‘rainbow pattern’ in Kaposi’s sarcoma

33.  The histopathological changes of KS typically
parallel the clinical progression of patch, plaque
and nodular stages.

34. Patch stage KS
• Clinical lesions consist of violaceous to brown erythematous
patches and plaques, most often involving the feet in classic
KS and often involving the face in AIDS‐associated KS

35. Histopathology – Patch stage
 Promontary
sign-
 Normal adnexal
structures and
preexisting
blood vessels
often protrude
into newly
formed blood
vessels

36. • horizontally arranged, irregular vessels that dissect in between
collagen bundles and around adnexae, vessels lined by
hyperchromatic plump endothelial cells, with areas of
erythrocyte extravasation & hemosiderin deposition.

37. Plaque stage KS
Well defines violaceous erythematous plaques

38. In the reticular
dermis-
spindle cell
vascular
proliferation
characterized
by slit-like
vascular
spaces with
extravasated
erythrocytes
Plaque stage

39. Plaque-stage Kaposi’s sarcoma. A more cellular dermis with
extensive hemorrhage.

40. Nodular KS
• Clinical lesions consist of well‐demarcated,
erythematous, firm nodules, often with accompanying
lymphoedema.

41. Nodular stage
Fairly well circumscribed dermal nodule with whorled
arrangement of spindled cells.

42. Angiomatous Kaposi’s sarcoma. Congested small vascular
channels in a focal sievelike pattern.

43. Lymphangiomatous Kaposi’s sarcoma. Numerous, irregular,
dilated, bloodless vascular channels with a striking
lymphangioma-like appearance.

44. Immunohistochemistry

45. Immunohistoche
mical staining -
antibodies
against latency-
associated
nuclear antigen
(LANA-1)
demonstrates
presence of
HHV-8

46. The blood vessels of
normal granulation tissue
(top) overlying an
ulcerated KS tumour
(bottom) are more
strongly immunoreactive
for CD31 than are KS
tumour cells.

47. nodular stage KS positive for CD31 is slightly less
than that of patch/plaque–stage KS, suggesting that
there may be subtle differences in the staining
patterns for endothelial markers between the
different histologic stages of KS.

48. CD34 demonstrates
strong
immunoreactivity in
KS spindle cells.
Sensitivity –
AIDS related KS –
100%
Non-AIDS related KS
– 100%

49. D2-40 stains all KS
lesional cells in this
cutaneous KS
nodule
Sensitivity –
AIDS related KS –
100%
Non-AIDS related KS
– 92%

50. Differential diagnosis of KS
Depending on site, distribution & morphology of
lesions of KS in individual cases, the D/D can vary
from-
 Naevus
 Histiocytoma
 Cryptococcosis
 Histoplasmosis
 Leishmaniasis
 Pneumocystis lesions
 Dermatophytosis
 Angioma
 Bacillary
angiomatosis
 Pyogenic granuloma
 Melanoma

54. Management (local)
 KS may resolve spontaneously in
immunocompetent individuals.
 Radiotherapy
 Cryotherapy
 Laser
 Alitretinoin gel
 Photodynamic therapy

55.  Surgical excision
 Topical antivirals: cidofovir, docosanol
 Intralesional, e.g. IFN‐α, TNF‐α, vinca alkaloids
 Surgery, e.g. cautery, infrared coagulation

56. Early‐stage KS (T0 stage):
• cART
• Local radiotherapy or liposomal anthracycline for
rapidly progressing or cosmetically disfiguring
disease
Advanced‐stage KS (T1 stage):
• cART & liposomal anthracycline
(daunorubicin 40 mg/m² every 14 days or
doxorubicin 20 mg/m² every 21 days)
Anthracycline‐refractory KS:
• cART & paclitaxel (100 mg/m² every 14 days)
Management (generalized lesion)

59.  cART
 Isotretinoin
 Cidofovir
 Intravenous chemotherapy, e.g. anthracyclines such as
liposomal daunorubicin, liposomal doxorubicin, bleomycin,
paclitaxel, vincristine, etoposide
 Human chorionic gonadotrophin?
 Interleukin 4
 Imatinib mesylate (platelet‐derived growth factor/c‐kit
receptor inhibitor)
 Thalidomide, lenalidomide, Sirolimus

60. • INF-α2b can be used in management of AIDS-
associated Kaposi sarcoma.
• Dosing - 30 million IU/m2 subcutaneously or IM 3
times/week until disease progression or maximal
response has been achieved after 16 weeks.
Biologicals

61.  Ethel Cesarman, Blossom Damania, Susan E. Krown,
Jeffrey Martin, Mark Bower, and Denise Whitby Kaposi
sarcoma Nat Rev Dis Primers. 2019 Jan 31; 5(1): 9.
 Rook’s textbook of dermatology 9th edition
 Fitzpatrick’s Dermatology 9th edition
 IADVL 5th edition
 Bolognia 4th edition
 https://www.cancer.gov/types/soft-tissue-
sarcoma/hp/kaposi-treatment-pdq#section/all

62.  Eleonora Ruocco, Vincenzo Ruocco, Maria Lina
Tornesello, Alessio Gambardella, Ronni Wolf, Franco
M Buonaguro Kaposi's sarcoma: etiology and
pathogenesis, inducing factors, causal associations,
and treatments: facts and controversies Clin Dermatol.
2013 Jul-Aug;31(4):413-422
 William Kamiyango, Jimmy Villiera, Allison Silverstein,
Erin Peckham-Gregory, Liane R. Campbell, and Nader
Kim El-Mallawany Navigating the Heterogeneous
Landscape of Pediatric Kaposi Sarcoma Cancer
Metastasis Rev. 2019 Dec; 38(4): 749–758.

63. • Tuğçe Ertürk Yılmaz, Bengü Nisa Akay, Aylin Okçu
Heper Dermoscopic findings of Kaposi sarcoma and
dermatopathological correlations Australas J Dermatol
2020 Feb;61(1):e46-e53.
• S-T Cheng, C-L K Ke, C-H Lee, C-S Wu, G-S Chen, S
C-S Hu Rainbow pattern in Kaposi's sarcoma under
polarized dermoscopy: a dermoscopic pathological
study Br J Dermatol 2009 Apr;160(4):801-9.
• Carlos S. Restrepo, Santiago Martínez,2, Julio A.
Lemos, Jorge A. Carrillo, Diego F. Lemos, Paulina
Ojeda, Prakash Koshy Imaging Manifestations of
Kaposi Sarcoma Radiographics. 2006 Jul-
Aug;26(4):1169-85.

64.  Flavia G Nunes Rosado, Doha M Itani, Cheryl M
Coffin, Justin M Cates Utility of immunohistochemical
staining with FLI1, D2-40, CD31, and CD34 in the
diagnosis of acquired immunodeficiency syndrome-
related and non-acquired immunodeficiency
syndrome-related Kaposi sarcoma Arch Pathol Lab
Med. 2012 Mar;136(3):301-4.
 L Pantanowitz, C N Otis, B J Dezube
Immunohistochemistry in Kaposi's sarcoma Clin Exp
Dermatol. 2010 Jan;35(1):68-72.

65. Thank You