Skin nonmelanocytic tumor
Vascular tumors
Acquired tufted angioma


Topic Completed: 1 August 2016

Minor changes: 15 January 2021

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PubMed Search: Acquired tufted angioma

Joel Tjarks, M.D.
Sara C. Shalin, M.D., Ph.D.
Page views in 2020: 4,314
Page views in 2021 to date: 192
Cite this page: Tjarks J, Shalin SC. Acquired tufted angioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticacquiredangioma.html. Accessed January 18th, 2021.
Definition / general
  • Benign, acquired vascular tumor most often arising in infancy or early childhood
  • Rare; approximately 200 cases reported in literature to date
Essential features
  • Tufts of capillaries infiltrating the dermis and subcutaneous adipose tissue in a “cannonball” or lobular pattern
  • Considered to be on the same neoplastic spectrum as kaposiform hemangioendothelioma; some consider it to be the same entity
Terminology
  • Synonyms: Tufted angioma, Nakagawa’s angioblastoma, progressive capillary hemangioma, tufted hemangioma
Epidemiology
  • Most commonly affects children and young adults with no gender predilection
  • Rare cases have been reported in adults
  • May be seen in association with Kasabach-Merritt syndrome
  • Some cases have been associated with liver transplantation, pregnancy, healed herpes zoster sites, or vaccination sites
Sites
  • Commonly found on neck, shoulders and trunk
  • Cranial and facial lesions are uncommon
Clinical features
  • Erythematous, poorly defined mottled macules and plaques typically ranging from 2 - 5 cm
  • Usually solitary
  • May have overlying hypertrichosis
  • Slowly growing, spreading lesion
  • Spontaneous regression rarely occurs; most lesions persist
Case reports
Treatment
  • Surgical excision for small lesions; recurrences are common
  • Low dose aspirin
  • High dose steroids (intralesional or systemic)
  • Pulsed dye laser
  • Chemotherapy(vincristine)
Clinical images

Contributed by Mark R. Wick, M.D.


Images hosted on other servers:

Subcutaneous
nodule

Microscopic (histologic) description
  • Multiple, scattered lobules of small capillary type vessels with small oval to spindle shaped cells throughout the dermis and subcutaneous tissue imparting a “cannonball” or glomerular appearance
  • May have variable mitoses without nuclear atypia
  • Hemosiderin may be present; in contrast to pyogenic granuloma, inflammation is typically absent
  • In contrast to Kaposi sarcoma, no slit-like vascular spaces and no plasma cells
  • In contrast to kaposiform hemangioendothelioma, confined to skin and less infiltrative
Microscopic (histologic) images

Contributed by Manuel Valdebran, M.D. and Phil LeBoit, M.D.



Contributed by Joel Tjarks, M.D.



Images hosted on other servers:

Cannon ball distribution

Positive stains
  • CD31, CD34
  • D2-40 highlights surrounding dilated dermal lymphatics
Negative stains
Differential diagnosis
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