Kaporsi Sarcoma DR Mark

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KAPOSI

SACOMA
DR MARK MUTUMA
DEFINITION
• KS is a spindle cell tumor of endothelial cell origin

• Varies from mild mucocutaneous disease to extensive organ


involvement.

• Linked with human herpesvirus 8 infection (HHV-8)


Classification
1. Classic / sporadic or European Ks

2. African-Endemic KS

3. Iatrogenic Immunosuppression-Associated

4. HIV associated/ epidemic


1. Classical

• Elderly males (6th decade) of Eastern Europe

• Legs – main, lymph nodes & abdomen

• Slowly progressive

• 10-15yrs survival

• 35% risk of secondary malignancy.


2. African Endemic
• Mainly in Zaire

• Young adults (35yrs) &young children (3yrs)

• Males> females

• Ass with not wearing shoes

• 5-8 yrs survival for adults and 2-3 yrs for children.
3. Immunosuppression-
Associated

Rare

• >Solid organ transplant recipients

• Chronic Rx with immunosuppressants

• 16.5 mnths after transplant

• Resolves with cessation of immunosuppression.


4. HIV Associated
K.S
• >Homosexual

• Rapid progressive

• Extensive systemic
involvement

• Survival - CD4 count &


opportunistic dses.
PATHOGENESIS
• Excessive proliferation of spindle cells from endothelial origin in
the background of reticular & collagen fibers and mononuclear
cells.

• This may result from stimulation of growth factors by HHV-8


Transmission
• Saliva
• Sexual transmission
• Blood / transfusion
• Organ transplant
Distribution

Widespread or localized

• Classic K.S. -on feet, legs & hands (Spread


centripetally)
-tip of nose,periobital areas, ear,scalp,penis.
-Rare on trunk

HIV related- Early face involvement


-Trunk invovment.
CLINICAL
a) SKIN LESIONS
Begin as ecchymotic-like macules

Evolve to patches, papules, plaques,nodules and


tumors.

Are red, pink or purple

Most - palpable (firm to hard)

Oval initially
• Secondary changes :
Erosion
Ulceration
Crusting
Hyperkeratosis

• Lymphydema –lower extremity(confluent of masses of lesion


due to deeper lymphatic involvement)
Mucous membranes

• Oral lesions- 1st in 22%

• Hard palate – violaceous stain, evolve to papules and


nodules with cobble stone appearance

• On soft palate, uvula, pharynx, gingiva &tongue.


Lab
• Skin biopsy-Diagnostic

• Imaging- Internal organ involvement.


MANAGMENT

• Classic KS- Radiotherapy

• Symptomatic African Endemic- Chemotherapy

• Immunosuppression ass- Regress or discontinue drug

• HIV related – Chemotherapy & HAART


• Local therapy on cometic,bulky,bleeding or functional
disturbing:
Cryosurgery

Laser surgery

Electrosurgery

Excisional surgery
DDX
• Hemangioma

• Eccymosis

• Insect bite rxn

• Melanocytic nevus

• Dermatofibroma

• Pyogenic granuloma

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