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Soft tissue tumors
Low / intermediate grade vascular tumors
Kaposi sarcoma
Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 24 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.
See subtypes below:
AIDS-associated,
chronic,
lymphadenopathic,
transplant-associated
General
=========================================================================
● Vascular proliferative disorder mediated by inflammatory cytokines and angiogenic growth factors in patients with HHV8 / Kaposi sarcoma associated herpesvirus infection, influenced by immune status
● May originate from cell type capable of undergoing lymphatic differentiation based on D2-40 staining, a lymphatic specific marker
(Mod Pathol 2002;15:434)
● Usually limited to skin; may involve mucus membranes, visceral organs, lymph nodes
● HHV8 also positive in multicentric Castleman’s disease, primary effusion lymphoma, some multiple myeloma
Micro images
=========================================================================
|
VEGFR3+ (fig C/D) |
D2-40+ |
Positive stains
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● FLI-1 (nuclear stain,
Am J Surg Pathol 2001;25:1061),
vascular endothelial growth factor receptor-3 (● Mod Pathol 2000;13:180), D2-40, other vascular markers
Differential diagnosis
=========================================================================
● Angiosarcoma: may have Kaposi-like features but is HHV8 negative (Arch Pathol Lab Med 2002;126:191)
● Kaposiform hemangioendothelioma: may have Kaposi-like features but is HHV8 negative
AIDS associated (epidemic)
General
=========================================================================
● Historically, 40% of homosexual men with AIDS got Kaposi vs. 5% of others with AIDS
● Incidence of Kaposi has been decreasing over time (Arch Pathol Lab Med 2002;126:182)
● Early involvement of lymph nodes and gut and wide dissemination
● Usually not a direct cause of death, although 1/3 develop lymphoma or another second malignancy
Terminology
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Macule / patch:
● Pink-purple macules of lower extremity or feet
● Superficial or mid-dermal proliferation of collagen-dissecting jagged capillary vessels with inconspicuous spindle cell component
● May be confluence of vessels
Plaque:
● Dermal, dilated, jagged vascular channels that dissect collagen fibers and contain isolated or small groups of spindle cells
● Red blood cell extravasation prominent
● Also hemosiderin laden macrophages, pink hyaline globules
Nodule / tumor:
● More distinctly neoplastic, most of lesion composed of spindle cells with intersecting fascicle like pattern in a background of inflammatory cells and red blood cells
● Small vessels and slitlike spaces with hyaline droplets and rows of red blood cells
● Mitotic figures common
● May involve lymph nodes and viscera (African and AIDS variants)
Clinical images
=========================================================================
Rapidly growing nodule |
Various images |
Gross description
=========================================================================
Indolent disease has 3 stages:
● Early - macule/patch
● Intermediate - plaque
● Late - nodule/tumor
Micro description
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● Dilated irregular blood vessels in background of lymphocytes, plasma cells, macrophages
● Resembles granulation tissue
● Disease spreads proximally, converts to raised
Micro images
=========================================================================
Various stains
Positive stains
=========================================================================
● Smooth muscle actin, D2-40 (Mod Pathol 2002;15:434), other vascular markers
Negative stains
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● Factor 8
Molecular / cytogenetics description
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● Detect HHV8 by PCR or in-situ hybridization
Chronic / classic
General
=========================================================================
● Classic type seen in Europeans
● Described by Kaposi in 1872
● 90% occur in older men from Eastern Europe, often Ashkenazic Jews; rare in US
● Associated with second malignant tumor or altered immune state, but not with HIV
● Multiple red-purple skin plaques or nodules in distal lower extremity, slowing increasing in size and spreading proximally
● Locally persistent with remission and relapses, but usually stay localized to skin
Case reports
=========================================================================
● 11 year old boy with maculopapular and nodular lesions on dorsum of hands and feet
(Indian J Dermatol Venereol Leprol 2011;77:730)
● 74 year old man with multiple, small, violaceous papules and nodules on trunk and extremities
(J Dermatol Case Rep 2012;6:52)
● 82 year old man with painful purple lesions on palms that worsened with cold
(Dermatol Online J 2011;17:16)
● 86 year old Mediterranean man with asymptomatic lesions on legs
(CMAJ 2011;183:E1040)
Clinical images
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|
|
|
|
Micro images
=========================================================================
Various images |
|||
11 year old boy |
74 year old man |
||
Figure 3a |
Figure 3b |
Figure 3c |
Figure 3d |
| Dermis is packed by fusiform cellular proliferation with some hyaline globules (Fig 3a and 3c), with areas of irregular cleft-like vascular channels lined by plump, slightly atypical and hyperchromatic endothelial cells (Fig 3b). Cells were HHV8+ (Fig 3d) | |||
Lymphadenopathic
General
=========================================================================
● Also known as African endemic
● Occurs in young Bantu children in South Africa (same population gets Burkitt lymphoma)
● Presents with localized or systemic lymphadenopathy
● Extremely aggressive disease; rarely is restricted just to lymph nodes; skin involvement is unusual
Transplant-associated
General
=========================================================================
● Occurs months to years after high-dose immunosuppressive therapy; 0.2-1.0% of kidney transplants
● Skin or metastatic lesions present
● Skin lesions may regress if immunosuppression is stopped
● Usually fatal if spreads to viscera
Case reports
=========================================================================
● 38 year old woman with purple lesion on leg (Saudi J Kidney Dis Transpl 2010;21:306)
● 40 year old man with severe hoarseness due to laryngeal involvement (Iran J Kidney Dis 2012;6:222)
● 49 year old man with ureteric lesion
(Exp Clin Transplant 2012;10:70)
Micro images
=========================================================================
49 year old man with ureteric lesion
End of Soft tissue tumors > Low / intermediate grade vascular tumors > Kaposi sarcoma
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