kaposi's sarcoma david lynch

7/29/2019 Kaposi's Sarcoma David Lynch

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Kaposis Sarcoma

June 2010

David Lynch,M4, CUMC

Deba P Sarma, MDOmaha


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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


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(No Immunosuppression!)

Medications:

allopurinal 100mg daily

diclofenac ophthalmic drops

diltiazem 180 mg daily

ferrous sulfate 325 twice dailylevothyroxine 75mcg daily

loratidine 10mg daily

metoprolol 200 mg bid

coumadin


4/27

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


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Derm consult for persistent papular rash

Has been itchy in the past which responded to

Lidex topical cream

(corticosteroid)


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4mm punch biopsy taken

at a depth of 5mm

Rule out vasculitis, amyloid


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Lets see the slides

Features of Kaposi Sarcoma

Bland thin walled vascular spaces

vs angiosarcoma

Spindle cell proliferation

Inflammation

Lymphocytes, macrophages, plasma cells


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H & E

Proliferative vascular neoplasm involving entire dermis


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Abnormal spindle cell proliferation with vascular slits and vascular

structures with red cell extravasation


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Special Stains

CD31

CD34

Factor VIII

HHV-8 antigen

Ki-67

SMA

CD68

S100


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CD 34: Positive


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HHV 8: Positive stippled dots in the nuclei


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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


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Kaposi Sarcoma Classification

Immunosuppression

Less risk from congenital immunosuppression

Iatrogenic carries highest risk

Organ transplant

AIDS

Typically aggressive

Far more common in MSM


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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


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HHV-8

Herpes virus

Latent and lytic phase

Unclear transmission route

Possibly sexual

Clearly increased in MSM


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HHV-8: What does it do?

Mild flu-like symptoms, or asymptomatic

Involved in malignancies

Kaposi

Multicentric Castlemans Disease

Primary Effusion Lymphoma


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Risk Factors for Classis KS

HHV-8 DNA

Location

Male Non-smoker

Immunosuppression

Including topical steroids! Chronic edema


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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


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Differential Diagnosis

Bacillary angiomatosis

Bartonella hensleae

Angiosarcoma

Pyogenic granuloma

S. schenckii

M. marinum

Hemangiomas


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How to Diagnosis

Vascular Markers

CD31

CD34

Factor VIII

HHV-8

Warthin-Starry silver stain for Bartonella

Endothelial cell atypia in angiosarcoma


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Treatment

In classic KS, treatment is not firmly

established

May not be necessary in the elderly!

Surgery

Chemotherapy

Radiation therapy


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To conclude

Features of Kaposi Male dominated

Thin, bland vascular structures, spindle cell

proliferation

Vascular marker and HHV8 +


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Special thanks to Dr. Sarma!


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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 InfectDis Rep (2010) 12: 147-154.

Elder, David, et al. Levers Histopathology of the Skin, 10th Ed. 2009.

kaposi's sarcoma david lynch

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