Kaposi sarcoma

1
Kaposis Sarcoma

• MD5 lecture
• Prof. NAA Mbembati

2
Introduction Kaposis sarcoma.

• First described by Moritz Kaposi(1872).
• Arises from vascular channels and perivascular
  connective tissues.
• A multifocal/multicentric disease.
• Currently classified into
• Classic
• endemic
• epidemic(AIDS_related) types.
• Iatrogenic/Immunosuppression associated K/S
• Epidemic type more common.

3
Clasic kaposis sarcoma

• Tends to occur around the Mediterranean Region
• Affects old men
• An indolent non-aggressive disease
• Not HIV related
• Lesions in hands and feet

4
Endemic Kaposis Sarcoma.

• Indolent disease.
• Peak age about 45 years.
• Malefemale ratio shows regional variation
  average 101.
• Aetiology unknown ?EBV, ?trauma (walking bare
  footed)
• HHV-8(Human herpes Virus type 8
• KSHVKaposi sarcoma associated herpes Virus

5
K.S. Clinical PresentationClassic and endemic
types

• 5 types of clinical lesions
• Nodular type
• Plaques
• Lymphadenopathic type
• Visceral type
• Florid type.

6
.
.
.

• Gross appearance of different Kaposi Sarcoma
  lesions including multicentric patch and nodular
  cutaneous and oral mucosal lesions.

7
K.S. Clinical Presentation(ctd)

• Nodular type
• The commonest type
• Nodules intradermal or subcutaneous.
• Onsetpins/needles sensationcoldness
• Oedema pittinggtwoody, then nodules appear.
• Nodules elaborate emptying sign

8
K.S. Clinical Presentation (ctd)

• Plaques
• Flattened hyperpigmented skin lesions
• Florid lesions
• Large granulomatous easily bleeding lesions.
• Lymphadenopathic type
• Predominantly in children, Poor prognosis.

9
K.S. Clinical Presentation(ctd)

• Visceral type
• May involve any organ eg GIT, lungs
• Poor prognosis
• Bone involvement may occur esp. in feet
• Cystic lesions
• Uniform rarefaction
• Complete bone destruction.

10
Epidemic Kaposis sarcoma.

• AIDS related disease.
• Type of lesions same as in endemic KS
• May involve any organ in the body
• More aggressive with rapid progression
• Affects younger ages peaks M-35yrs, F-28yrs
• Extensive disease with multiple lesions
  co-existingextensive oedema

11
Epidemic kaposis sarcoma

• AIDS associated
• Centrifugal distribution involving thighs, legs
  face, trunk, vulva,oral mucosa
• Lymphadenopatic type common even in adults
• Often have widespread visceral disease.
• High morbidity and mortality
• Death usually from opportunistic infections

12
Iatrogenic K/S

• The KS associated with immunosuppressive The
  clinical and morphological aspects are similar to
  the classic form.
• The suspension of the immunosuppressive treatment
  usually causes the regression of the lesions, but
  increases the risk of losing the transplant.

13
Pathogenesis

• Various cofactors have been implicated in the
  pathogenesis of KS, including genetic
  susceptibility, immunologic alterations,
• Human herpes virus 8 (HHV-8) also known as Kaposi
  sarcoma-associated herpes virus (KSHV) is
  believed to be a major causative factor for all
  clinical variants of KS.

14
Histology

• Histology Spindle cells with vascular
  slitsmononuclear cells around blood vessels.
• Predominance of endothelial cells
• Hisological types
• Mixed- equal amount of spindle cells, vascular
  clefts and capillaries
• Monomorphic- one cell type predominating
• Anaplastic-.cellular pleomorphism and mitotic
  figures

15
K.S. Diagnosis and Differential Diagnosis

• Depends on presentation eg for lymphadenopathic
  and nodular lesions-
• Other causes of LN enlargement
• Nodular Leprosy
• Madura mycosis-(Madura foot)
• Neurofibromatosis.
• Investigations
• Lymph node or skin biopsy
• CXR, FBP, ELISA for HIV,
• CT scan in selected cases with visceral
  involvement
• Endoscopy in GIT manifestations

16
K.S. Treatment

• Antiretroviral therapy
• Treat opportunistic infections
• Radiation therapy for localised disease
• ?Surgery-
• Badly damaged extemities?

17
K.S. Treatment

• Chemotherapy eg.
• Vincristine
• Doxorubicin
• Immunotherapy
• Cessation of immunosuppressive therapy
• Epidemic type have shorter remission periods and
  recurrences are common,