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Penis and scrotum
Other malignancies
Kaposi’s sarcoma
Editor: Antonio Cubilla, M.D. and Alcides Chaux, M.D. (see Author/Reviewers page)
Revised: 19 May 2010, last major update May 2010
Copyright: (c) 2002-2010, PathologyOutlines.com, Inc.
Definition
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● Low grade vascular tumor caused by human herpesvirus 8 (HV8), also called Kaposi's sarcoma associated herpesvirus (KSHV), and usually associated with HIV
● ICD-O: 9140/3
Epidemiology
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● Classic: affects elderly men of Mediterranean origin and Ashkenazi Jews; presents as indolent disease with nodular lesions on skin, and affecting more often the lower limbs and feet and rarely with visceral involvement
● Endemic: young men in sub-Saharan Africa, more aggressive, affects skin extensively, particularly lower limbs; NOT related to HIV
● Transplant related: more prominent after use of cyclosporine for transplant patients
● Epidemic: associated with AIDS or less commonly other immunosuppressed states; patients younger than with other sarcomas
Sites
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● Affects distal penis, mainly glans and coronal sulcus but also foreskin and penile shaft
Etiology
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● HHV8 / KSHV detected in most cases
● Unclear whether it represents a true neoplasm or a reactive condition
Clinical features
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● Most common sarcoma of penis
● Blue-purple to erythematous lesions
● Frequently multicentric
● May present in any of its typical stages (patch, plaque or nodular)
Case reports
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● 44 year old bisexual man who was HIV negative (Archives 1986;110:346)
Treatment
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● Treatment for HIV (HAART); also local excision, cryotherapy, laser therapy or radiation therapy
● Also interferon alpha or chemotherapeutic drugs
Clinical images
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42 year old homosexual with ulcerated 36 year old HIV+ man with extensive
plaque in coronal sulcus involvement of glans causing meatal stricture
Tumor forms discrete nodules Nodules/papules in glans
Gross description (Macroscopy)
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● Usually multiple, small, 2-6 mm bluish lesions in glans
Micro description (Histopathology)
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Patch stage
● Subtle proliferation of jagged vessels separating collagen bundles
● Promontory sign: adnexal structures and preexisting vessels protruding into newly-formed vessels
● Extravasated erythrocytes and hemosiderin-laden macrophages
● Patchy inflammatory infiltrate composed of lymphocytes and plasma cells
Plaque stage
● Vascular proliferation is more evident
● Bland spindle cells appear between vessels
● Intra- and extracellular PAS+ hyaline globules
● Autolumination: paranuclear vacuoles containing erythrocytes
Nodular stage
● Spindle cells merge to form well-defined nodules
● Absence of significant nuclear atypia
● Slit-like vascular spaces with numerous erythrocytes
● Intracytoplasmic grape-like clusters of PAS+ diastase-resistant hyaline globules
● Prominent lymphoplasmacytic inflammatory infiltrate
Micro images
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Low power High power; note the slit-like blood vessels
Other sites
Patch stage with Patch stage
promontory sign
Plaque stage with hyaline globules Plaque stage
and autolumination (arrow)
Nodular stage; spindle cells Resembles cavernous
are HHV8+ (fig C) hemangioma
H&E and D2-40 Factor VIII+
Positive stains
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● CD34, CD31, Factor VIII related antigen, podoplanin (D2-40)
Negative stains
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● Desmin, cytokeratin
Electron microscopy images
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Lymph node in AIDS patient
Differential Diagnosis
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● Angioma: lacks jagged vascular pattern, absence of promontory sign, absence of evident inflammatory infiltrate
● Angiosarcoma: pleomorphic cells lining vascular spaces
● Fibrosarcoma: lacks slit-like vascular spaces containing erythrocytes
● Lichen sclerosus: nodules may appear post-steroid treatment that clinically resemble Kaposi's sarcoma (Dermatol Ther 2009;22:379)
● Sarcomatoid carcinoma: significant pleomorphism, positivity for pan cytokeratin, cytokeratin 34-beta-E12, p63
Additional references
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End of Penis and scrotum > Other malignancies > Kaposi’s sarcoma
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