Kaposi’s 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

Let’s 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 Castleman’s 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 Kaposi’s
• 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. Lever’s Histopathology of the Skin, 10th Ed. 2009.

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