Skin tumor - Nonmelanocytic

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Skin - Nonmelanocytic tumors

Vascular tumors

Hemangioma

Reviewer: Christopher Hale, M.D. (see Reviewers page)
Revised: 16 July 2012, last major update July 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● Childhood tumors are often malformations, not neoplasms

Capillary hemangioma

General
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● Small (usually 1-3 mm) benign vascular lesions
● Also called strawberry hemangioma or Campbell de Morgan spot
● Children: usually regress by fibrosis
● Adults: may slowly enlarge or thrombose

Differential diagnosis
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● Dermal vascular hyperplasia (with venous stasis)
● Pyogenic granuloma
● Kaposi sarcoma
● Angiosarcoma
● Hemangioendothelioma

Cavernous hemangioma

General
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● Markedly dilated dermal vessels, may elevate overlying epidermis, which may be atrophic
● Associated with Maffucci syndrome, blue rubber bleb nevus syndrome, Kasabach-Merritt syndrome

Epithelioid hemangioma

General
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● Also called histiocytoid hemangioma, angiolymphoid hyperplasia with eosinophilia (ALHE)
● All racial groups
● Head and neck nodules, often periauricular
● Benign in skin
● May be reactive
● May occur in bone
● No/rare regional lymphadenopathy
● Normal serum eosinophils, IgE

Case reports
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● 51 year old male with 8 × 6 cm subcutaneous mass in right cheek (Int J Surg Case Rep 2011;2:258)

Gross description
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● Small, superficial, dermal papulonodules, frequently erythematous, with bleeding

Micro description
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● Proliferation of blood vessels with epithelioid endothelial cells exhibiting abundant eosinophilic cytoplasm with variable cytoplasmic vacuoles resembling intracytoplasmic lumina and large vesicular nuclei with variable atypia
● Usually heavy infiltrate of eosinophils and lymphocytes with germinal centers
● May have lobular solid pattern

Micro images
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Aggregate of lymphoid tissue and numerous blood vessels

Blood vessels lined by cuboidal endothelial cells and numerous eosinophils in background

Differential diagnosis
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● Epithelioid hemangioendothelioma (usually not cutaneous)
● Epithelioid angiosarcoma (marked atypia)
● Lobular pyogenic granuloma (no epithelioid endothelial cells)
● Kimura’s disease (usually Asians with elevated serum eosinophils and IgE, usually regional lymphadenopathy)

Glomeruloid hemangioma

General
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● Associated with Castleman disease and POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes)

Case reports
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● 49 year old milkman with 6 mm purple nodule on anterior chest (Nephrol Dial Transplant 2001;16:2105)

Micro description
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● Dermal vascular spaces contain glomeruloid structures formed by capillaries
● Occasional cells have PAS+ eosinophilic globules

Micro images
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Contributed by Angel Fernandez-Flores, MD, PhD, Hospital El Bierzo and Clinica Ponferrada, Spain

Additional references
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● Am J Dermatopathol 2008;30:539, Clin Exp Dermatol 2009;34:800

Hobnail hemangioma

General
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● Endothelial cells protrude into vessel lumina

Case reports
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● 7 year old boy with dark brown papule with ecchymotic halo on left upper back (Ann Dermatol 2011;23:539)

Micro description
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● Proliferation of irregular dissecting vascular channels lined by plump endothelial cells

Micro images
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Contributed by Drs. Asmaa Gaber Abdou and Nancy Asaad, Menofiya University, Egypt - lower lip of female

Positive stains
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● CD31, CD34, D2-40

Negative stains
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● HHV8

Differential diagnosis
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● Kaposi's sarcoma (patch stage)
● Benign vascular tumors
● Progressive lymphangioma

Juvenile hemangioma

General
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● Usually congenital or perinatal and grow during the first few months of life
● Spontaneously involutes, not associated with Kasabach-Merritt phenomenon

Case reports
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● 3 year old male child with painless, dome-shaped lesion of the upper lip (J Oral Maxillofac Pathol 2011;15:316)

Clinical images
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Various images

Micro images
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Various images

Positive stains
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● GLUT1+, LewisY+

Differential diagnosis
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● Kaposiform hemangioendothelioma: doesn’t involute, GLUT1-, LewisY-, often associated with Kasabach-Merritt phenomenon

Microvenular hemangioma

General
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● Young to middle-aged adults
● Also pregnant women or women on oral contraceptives
● Small, enlarging, purple-red nodules or plaques of extremities
● Duration up to 4 years

Micro description
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● Transdermal proliferation of irregular branching venules with indistinct lumina, no atypia, no fat invasion (although may grow along collagenous septa of subcutis)
● Variable dermal fibrosis and lymphocytes
● Resembles acquired (tufted) angioma, stasis change, sclerosing hemangioma

Positive stains
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● Endothelial cells are strongly Ulex europaeus lectin 1+, weakly positive for factor VIII related antigen

Differential diagnosis
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● Dermatofibroma
● Kaposi’s sarcoma: irregularly anastomosing vascular spaces, plasma cells, hyaline globules, fascicles of spindle cells

Spindle cell hemangioma

General
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● Formerly called spindle cell hemangioendothelioma
● Associated with Mafucci syndrome

Case reports
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● 43 year old Japanese man with Maffucci syndrome (J Med Case Reports 2011;5:224)

Micro description
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● Bland spindle cell proliferations between vascular lumina with extravasated erythrocytes (similar to Kaposi’s sarcoma), but also with vacuolated cells and epithelioid endothelial cells (unlike Kaposi’s sarcoma)

Micro images
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Various images

End of Skin - Nonmelanocytic tumors > Vascular tumors > Hemangioma

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