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Soft tissue tumors
Low / intermediate grade vascular tumors
Hemangioendothelioma
Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 25 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.
See subtypes below:
composite,
epithelioid / histiocytic,
endovascular papillary,
kaposiform,
polymorphous,
retiform,
spindle cell
General
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● Intermediate grade vascular tumor with variable histologic features and clinical behavior
● 40% recur, 20% metastasize, 15% die of tumors
Positive stains
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● FLI-1 (nuclear stain,
Am J Surg Pathol 2001;25:1061)
Composite hemangioendothelioma
Definition
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● Mixtures of benign, low-grade malignant and malignant vascular components
● Predominant components resemble epithelioid and retiform hemangioendothelioma
● Very rare
● Usually women, median age 43 years, range 22-75 years
● Usually superficial dermis or subcutaneous, usually in hands or feet
● Recur locally, may metastasize, no deaths after median follow-up of 5 years
● Must sample extensively to obtain correct diagnosis
Case reports
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● 50 year old woman with dyspnea and cough
(Tumori 2009;95:98)
Treatment
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● Excision, may recur locally, but no/rare metastases
(Am J Surg Pathol 2007;31:1567)
Positive stains
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● CD31, CD34, Factor VIII
Additional references
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● Am J Surg Pathol 2000;24:352
Epithelioid / histiocytic hemangioendothelioma
General
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● Intermediate grade vascular malignancies closely associated with or arising from a vein in 50% of cases
● Usually adults, 60% women
● Extremities (60%); also head and neck, mediastinum, trunk
● Unpredictable clinical course, but less aggressive than angiosarcoma
● 13% recur, 20-30% metastasize (lung, lymph nodes), 13% die of disease
(Am J Surg Pathol 1997;21:363);
for lung, mortality is 65%
● High risk (> 3 MF/50 HPF and size > 3 cm) have 5 year disease specific survival of 59% versus 100% for low risk
(Am J Surg Pathol 2008;32:924)
Case reports
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● 16 year old girl with painful progressive swelling of lower limb
(Indian J Dermatol 2012;57:53)
● 24 year old man with forearm mass
(Diagn Pathol 2011;6:120)
● 27 year old woman with lower eyelid mass
(Oman J Ophthalmol 2011;4:142)
● 42 year old woman with tumor of sphenoid bone
(Neurol India 2012;60:344)
● 55 year old man with thigh mass
(Case of the Week #77)
Treatment
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● Low grade tumors: wide local excision
● High grade tumors: radical local excision with possible neck dissection (head & neck tumors)
Clinical images
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Forearm |
Lower eyelid |
Gross description
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● Variable size, up to 18 cm
Micro description
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● Cords or small nests of round endothelial cells with abundant eosinophilic cytoplasm
● Tumors arising from vessels extend outward from the lumen towards soft tissue
● Tumor cells often have intracytoplasmic vacuoles representing small vascular lumina, which may resemble mucin
● Nuclei are round and may be indented
● Usually minimal mitotic activity, atypia or necrosis, but 25% of cases exhibit frank malignant features of prominent nuclear pleomorphism, mitotic activity, focal spindling or necrosis
● Stroma may be scanty or myxoid
● May have peripheral inflammatory infiltrate with germinal centers and eosinophils, multi-nucleated giant cells
Micro images
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Cytology description
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● Clusters of polygonal cells with moderate nuclear atypia
(J Cytol 2012;29:89)
Cytology images
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Polygonal cells with moderate atypia
Positive stains
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● Vimentin, CD31, von Willebrand factor, keratin (30%, focal), reticulin (nests and cords of cells are invested by a reticulin sheath)
Molecular / cytogenetics description
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● Occasional tumors may demonstrate t(1;3)(p36.3;q25,
Am J Surg Pathol 2001;25:684)
Differential diagnosis
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● Epithelioid angiosarcoma: irregular sinusoidal vascular channels, solid sheets of cells with marked atypia and prominent mitotic activity, necrosis
● Epithelioid sarcoma: distal extremities of young adults, tumor cells merge with collagenous stroma, keratin+ (strong), CD31-
● Melanoma: S100+, HMB45+, CD31-
● Metastatic carcinoma: more marked atypia, mitotic activity, usually not angiocentric, keratin+, CD31-
Endovascular papillary hemangioendothelioma
General
=========================================================================
● Also known as Dabska's tumor
● Very rare tumor of children in skin or soft tissue
● Good prognosis, with only rare nodal metastases
Case reports
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● 10 month old boy with pain and tender scrotal swelling
(Diagn Pathol 2006;1:12)
Gross images
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Testicular tumor
Micro description
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● Papillary tufts lined by plump endothelial cells (epithelioid- or histiocytic-like) within dilated vascular lumina
● May have glomeruloid appearance
Micro images
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Testicular tumor |
VEGFR3+ |
|
Positive stains
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● Vascular endothelial growth factor receptor 3 (Mod Pathol 2000;13:180), FLI1 (Am J Surg Pathol 2001;25:1061)
Kaposiform hemangioendothelioma
General
=========================================================================
● Rare, locally aggressive; tumor of infants and children; affects skin (75%), retroperitoneum (18%), bone
● Death due to extensive disease and severe coagulopathy (Kasabach-Merritt syndrome), although no metastatic potential (Mod Pathol 2001;14:1087)
● Usually initial tumor is cutaneous
Case reports
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● 2 year old boy with tonsillar mass (World J Surg Oncol 2011;9:57)
Clinical images
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2 year old boy with tonsillar mass
Micro description
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● Infiltrating nodules and sheets of compact spindle cells with slit-like lumina
Micro images
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Positive stains
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● Vascular endothelial growth factor receptor-3 (Mod Pathol 2000;13:180)
Differential diagnosis
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Polymorphous hemangioendothelioma
General
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● <10 cases reported
● Lymph nodes and soft tissue
● Recurs locally, rare metastases
Micro description
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● Combinations of solid, primitive vascular and angiomatous patterns
● Uniform cytologic features
● No epithelioid, spindle cell or angiosarcoma-like areas
Micro images
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Patterns in same tumor
Left: kaposiform pattern with extravasated red blood cells and blood cells within neolumina; mild atypia, no mitotic activity
Middle: epithelioid (lower right) and spindle patterns (upper left) with intracytoplasmic vacuoles containing erythrocytes
Right: CD34+
Typical mixed solid and angiomatous features
Retiform hemangioendothelioma
General
=========================================================================
● Low grade variant of angiosarcoma
● Usually distal extremities of young individuals
● Weiss and Goldblum use term “hobnail hemangioendothelioma” for retiform and Dabska-type tumors, which they believe to be closely related
● Rarely multiple
(Am J Dermatopathol 1996;18:606)
● 2/3 recur, particularly without wide local excision; low rate of metastases, no tumor related deaths
Case reports
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● 20 year old man with mass in middle phalynx of finger
(Arch Plast Surg 2012;39:80)
● 37 year old man with skin lesion on back
(Case of the Week #107)
● 44 year old man with thigh mass
(Sarcoma 2010;2010 pii: 756246)
● 61 year old woman with painful lesion on scalp
(Int J Clin Exp Pathol 2010;3:528)
Treatment
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● Wide local excision
Clinical images
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Gross description
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● Lesion of reticular dermis and subcutaneous tissue
Micro description
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● Retiform (net-like, similar to rete testis) pattern of blood vessels that disperse through reticular dermis and subcutis
● Vessels lined by monomorphic hobnail endothelial cells with scant cytoplasm and rounded, naked-type nuclei
● Often prominent lymphocytic infiltrate
● No epithelioid areas or cytoplasmic vacuoles
(Am J Surg Pathol 1994;18:115)
Micro images
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Positive stains
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● Endothelial cells: CD34 (strong), CD31, vWF
Negative stains
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● Endothelial cells: keratin
Differential diagnosis
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● Angiosarcoma: may focally have low grade features, but also exhibits areas of marked atypia and pleomorphism; also dissects between individual collagen bundles and has mitotic activity
● Hobnail hemangioma: smaller, more superficial and more localized, with papillary dermal vessels that disappear into reticular dermis
Spindle cell hemangioendothelioma
General
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● Any age, usually males, usually distal extremities
● Low grade lesion which recurs commonly and may be multicentric, but only one reported metastases after repeated recurrence and radiation therapy
● May be a hamartoma due to aberrations in local blood blow; perhaps should be called spindle cell hemangioma
● Associated with Mafucci's syndrome
Case reports
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● 67 year old woman with swelling in temporal region
(Case Rep Surg 2011;2011:481654)
Clinical images
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Intramuscular tumor of temporal region |
Gross description
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● Dermal or subcutaneous tumor
Micro description
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● Cavernous hemangioma and Kaposi sarcoma like features
● Cavernous spaces with solid areas composed predominantly of bland spindle cells, with a minor component of epithelioid, often vacuolated, endothelial cells, usually associated with irregular fascicles of smooth muscle fibers and adjacent malformed vessels
Micro images
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Intramuscular tumor of temporal region
Various images
Positive stains
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● Endothelial markers
End of Soft tissue tumors > Low / intermediate grade vascular tumors > Hemangioendothelioma
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