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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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Forearm mass
Spine
Lower eyelid
Sphenoid bone
Case of the Week #77: thigh tumor - H&E and CD31

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
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● 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
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● 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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Glomeruloid nests of spindled and epithelioid cells
"Cannon ball" appearance
Slit-like lumens
VEGFR3+
Various images
Tonsillar mass

Positive stains
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● Vascular endothelial growth factor receptor-3 (Mod Pathol 2000;13:180)

Differential diagnosis
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Kaposi's sarcoma


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
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● 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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Finger
Scalp

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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Finger
Scalp


Case of Week #107: H&E and CD31

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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