Soft tissue tumors - Microvenular hemangioma

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Soft tissue tumors

Benign vascular tumors

Microvenular hemangioma

Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 24 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

Definition
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● Dermal proliferation of small, irregular branching capillaries and venules with inconspicuous lumina

Terminology
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● First described in 1989 as microcapillary angioma (Z Hautkr 1989;64:1071), and in 1991 with current terminology (J Cutan Pathol 1991;18:235)

Epidemiology
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● Rare, < 50 cases reported
● Young to middle aged adults

Sites
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● Often trunk or limbs

Etiology
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● May be form of acquired venous hemangioma
● Microcapillary hemangioma: similar histology, but occurs in young women taking oral contraceptives or during pregnancy

Clinical features
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● Presents as slow growing, solitary, asymptomatic, purple-red papule or plaque, 0.5-2 cm, usually extremeties
● May mimic an inflammatory lesion (J Plast Reconstr Aesthet Surg 2009;62:e166)

Case reports
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● Boy with coexisting AML (Pediatr Dermatol 2003;20:266)
● 23 year old woman with slowly growing, solitary red plaque on back (Pathol Int 1998;48:237)
● 28 year old man with thigh nodule (Case of the Week #80)
● 40 year old woman (Dermatology 2003;206:161)
● 53 year old woman with numerous bilateral macules, patches, and plaques (Am J Dermatopathol 2013;35:98)
● 55 year old woman with POEMS syndrome and HHV8+ microvenular hemangioma (Arch Pathol Lab Med 2003;127:1034)

Treatment
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● Excision is curative

Dermoscopic description
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● Multiple well-demarcated red globules; fine pigment network at periphery (Dermatology 2007;215:69)

Gross images
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Well defined, oval red lesion

Microscopic description
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● Dermal proliferation of small, irregular branching capillaries and venules with inconspicuous lumina
● Endothelial cells may be plump, but no atypia
● Stroma is collagenous
● Variable lymphocytes
● No fat invasion, although may grow along collagenous septa of subcutis
● No spindle cells

Micro images
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Case of the Week images

Dermal vascular proliferation

HHV8+ tumor

Positive stains
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● Endothelial cells: CD34, CD31, Factor VIII related antigen, vWF, Ulex europaeus lectin
● Pericytes: smooth muscle actin

Negative stains
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● Podoplanin (Pathol Res Pract 2008;204:817), HHV8

Differential diagnosis
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● Acquired (tufted) angioma: multiple vascular lobules similar to pyogenic granuloma but more cellular, resembling cannonballs
● Kaposi's sarcoma-patch stage: irregular vascular spaces are anastomosing but not collapsed, and are accompanied by atypical endothelial cells, eosinophilic hyaline globules, plasma cells and fascicles of spindle cells; may be irregular dissection of collagen bundles by vessels; spindle cells are HHV8+, patients are HIV+ (Am J Clin Pathol 2004;121:335)
● Kaposiform hemangioendothelioma: also has slit-like lumina, but they are due to nodules and sheets of compact spindle cells; affects the skin or retroperitoneum of infants and children, may be associated with severe coagulopathy
● Sclerosing hemangioma
● Statis related change

End of Soft tissue tumors > Benign vascular tumors > Microvenular hemangioma

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