Contents

SynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences

View all Images (39)

Angiosarcoma of skin
See also in: Cellulitis DDx,Hair and Scalp
Other Resources UpToDate PubMed

Angiosarcoma of skin

See also in: Cellulitis DDx,Hair and Scalp
Contributors: Annie Chen BS, John Zampella MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Angiosarcomas are rare, aggressive, malignant neoplasms of vascular endothelial cell origin. They may arise in any organ or body site but have a predilection for skin and superficial soft tissues.

The most common form of angiosarcoma is cutaneous angiosarcoma (cAS). This may be primary, in which case it is most frequently located on the head or neck, particularly the scalp, of older individuals, more frequently men (ratio of 2:1); or secondary. In secondary cases, cAS develops in sites of chronic lymphedema or radiation exposure, and this subtype may be seen in a younger population. This type of cAS has also been termed lymphangiosarcoma.

Angiosarcomas grow rapidly, recur locally, and metastasize widely. Five-year survival rates of primary cAS range from 11%-50%. This is due to the biologic behavior of the tumor, delays in diagnosis, and poor impact on survival with currently available treatment options.

The appearance of angiosarcoma is highly variable. It may mimic ecchymosis or hematoma, cellulitis, rosacea, or facial edema, and this may lead to delay in diagnosis. The tumor or plaque may bleed or ulcerate after minor trauma and grow rapidly. Satellite nodules may be present.

Symptomatic thrombocytopenia (Kasabach-Merritt syndrome) has been seen in cases of angiosarcoma.

Pediatric patient considerations: Angiosarcoma is exceedingly rare in children and is more likely to be visceral than cutaneous. The prognosis is poor.

Codes

ICD10CM:
C49.9 – Malignant neoplasm of connective and soft tissue, unspecified

SNOMEDCT:
254794007 – Angiosarcoma of skin

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

  • Traumatic purpura / hematoma
  • Cellulitis or Erysipelas
  • Rosacea
  • Angioedema
  • Rosacea
  • Other vascular tumors, both benign and malignant, including Non-AIDS Kaposi sarcoma
  • Sarcoidosis
  • Leukemia cutis
  • Lymphoma
  • Metastatic cutaneous carcinoma or Metastatic cutaneous melanoma
  • Dermatofibrosarcoma protuberans
  • Cutaneous leiomyosarcoma
  • Intravascular papillary endothelial hyperplasia
  • Angiolymphoid hyperplasia with eosinophilia
  • Cutaneous squamous cell carcinoma
  • Primary Amelanotic melanoma
  • Atypical fibroxanthoma
  • Epithelioid sarcoma
  • Vasculitis
  • Ecthyma gangrenosum
  • Angioinvasive fungal disease such as Aspergillosis

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:08/16/2023
Last Updated:09/24/2023
Copyright © 2024 VisualDx®. All rights reserved.
Angiosarcoma of skin
See also in: Cellulitis DDx,Hair and Scalp
A medical illustration showing key findings of Angiosarcoma of skin : Forehead, Frontal scalp, Smooth plaque, Tumor, Ecchymosis
Clinical image of Angiosarcoma of skin - imageId=977208. Click to open in gallery.  caption: 'An extensive reddish and brownish plaque on the scalp and forehead.'
An extensive reddish and brownish plaque on the scalp and forehead.
Copyright © 2024 VisualDx®. All rights reserved.