Kaposis Sarcoma

June 2010 David Lynch,M4, CUMC Deba P Sarma, MD Omaha

Case
HPI: MR is an 85 year old female admitted for atrial fibrillation with RVR
PMH: breast cancer s/p lumpectomy and radiation Afib, HTN, stasis edema, anemia, hypothyroid

Medications: allopurinal 100mg daily diclofenac ophthalmic drops diltiazem 180 mg daily ferrous sulfate 325 twice daily levothyroxine 75mcg daily loratidine 10mg daily metoprolol 200 mg bid coumadin (No Immunosuppression!)

 Family Hx: positive for CAD Social: Non-smoker, no alcohol, no drugs PE: 99.3 123 131/81 14
Purple infiltrated papules plus macules in both legs below the knee Chronic 5 cm papular / purpuric

 Derm consult for persistent papular rash

Has been itchy in the past which responded to Lidex topical cream
(corticosteroid)

 4mm punch biopsy taken at a depth of 5mm

Rule out vasculitis, amyloid

Lets see the slides

Features of Kaposi Sarcoma
Bland thin walled vascular spaces
vs angiosarcoma

Spindle cell proliferation Inflammation

Lymphocytes, macrophages, plasma cells

H&E

Proliferative vascular neoplasm involving entire dermis

Abnormal spindle cell proliferation with vascular slits and vascular structures with red cell extravasation

Abnormal spindle cell proliferation with vascular slits and vascular structures with red cell extravasation

Abnormal spindle cell proliferation with vascular slits and vascular structures with red cell extravasation

Special Stains
CD31 CD34 Factor VIII HHV-8 antigen Ki-67 SMA CD68 S100

CD 34: Positive

HHV 8: Positive stippled dots in the nuclei

Kaposi Sarcoma Classification

Classic
Older men
15:1 men to women > 50 years old

Occurs on legs, indolent course

Endemic
African children and young adults 3:1 male to female more aggressive course than classic Most common tumor in Uganda in 1960s

Kaposi Sarcoma Classification

Immunosuppression
Less risk from congenital immunosuppression Iatrogenic carries highest risk
Organ transplant

AIDS
Typically aggressive Far more common in MSM

CKS Epidemiology
Men > women Most common in Mediterranean or Europe HHV-8
HHV-8 seroprevalence varies
2% North America 20% in Italy 25% HIV positive Americans

HHV-8
Herpes virus
Latent and lytic phase

Unclear transmission route

Possibly sexual Clearly increased in MSM

HHV-8: What does it do?

Mild flu-like symptoms, or asymptomatic Involved in malignancies
Kaposi Multicentric Castlemans Disease Primary Effusion Lymphoma

Risk Factors for Classis KS

HHV-8 DNA Location Male Non-smoker Immunosuppression
Including topical steroids!

Chronic edema

Progression of the Kaposis

Macular stage
Sparse dermal involvement

Plaque
Diffuse dermal involvement

Nodular stage
Honeycomb of bland thin walled vascular spaces Back to back vessels not seen in angiosarcoma Endothelial cells are bland

Differential Diagnosis
Bacillary angiomatosis
Bartonella hensleae

Angiosarcoma
Pyogenic granuloma S. schenckii M. marinum Hemangiomas

How to Diagnosis
Vascular Markers
CD31 CD34 Factor VIII

HHV-8 Warthin-Starry silver stain for Bartonella Endothelial cell atypia in angiosarcoma

Treatment
In classic KS, treatment is not firmly established May not be necessary in the elderly!
Surgery Chemotherapy Radiation therapy

To conclude
Features of Kaposi
Male dominated Thin, bland vascular structures, spindle cell proliferation Vascular marker and HHV8 +

 Special thanks to Dr. Sarma!

References
Uptodate. Classic Kaposi's sarcoma: Epidemiology, risk factors, pathology, and molecular pathogenesis. Accessed 27 June 2010. Uptodate. Epidemiology and transmission of human herpesvirus 8 infection. Accessed 28 June 2010 Sunil, Meena, et al. Update on HHV-8 Associated Malignancies. Curr Infect Dis Rep (2010) 12: 147-154. Elder, David, et al. Levers Histopathology of the Skin, 10th Ed. 2009.