Skin - Nonmelanocytic tumors
Vascular tumors
Acquired tufted angioma

Author: Joel Tjarks, M.D. (see Authors page)
Editor: Sara Shalin, M.D., Ph.D.

Revised: 24 August 2016, last major update August 2016

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Acquired tufted angioma

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

Images hosted on PathOut servers:

Courtesy of Mark R. Wick, M.D.



Images hosted on other servers:

Wide tufted angioma
and Kasabach-Merritt
syndrome in patient
11 at age 6 months

Various images

Micro 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
Micro Images

Images hosted on PathOut servers:

Courtesy of Joel Tjarks, M.D.



Courtesy of Mark R. Wick, 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