Angiosarcoma

Question 1

What is an angiosarcoma?

Answer 1

Uncommon and aggressive tumour of endothelial cells

These cells line BV and Lymphatics

Question 2

Clinical features of an angiosarcoma

Answer 2

Elderly patients
50% involve the head and neck

Bruis like patch with odema, becomes a violaceous plaque / nodule

Bleed easily
Ulcerate
Centrifugal spread

Question 3

Prognosis of angiosarcoma

Answer 3

Poor prognosis
Highly aggressive
Recurrence rate and metastatic disease rate is still high even with negative margins
less than 15% survival over a 5-year period

Question 4

RFs for Angiosarcoma

Answer 4

Elderly
Male

Chronic lymphedema
- typically post BC and LNB disection (Stewart Treves syndrome) - occuring on the upper arm

Previous radiotherapy at the site

Environmental exposures:
- arsenic
- thorium dioxide
- radium
- vinyl chloride

Question 5

Histoogy of angiosarcoma

Answer 5

Angiosarcoma has a wide morphologic appearance,

Numerous irregularly shaped anastomosing vascular channels lined by atypical endothelial cells with a highly infiltrative architecture and poor demarcation

Tumor cells are typically plump, pleomorphic and mitotically active
They can be spindle shaped, polygonal, epithelioid and primitive round cells, forming papillae or solid nests within vascular lumina

• Less well differentiated – endothelial cells show more pronounced nuclear pleiomorphisms and mitotic activity pile up and form papillary projections
• Nodular areas commonly epithelioid with more atypia
• In poorly differentiated areas – lumina formation may be non-apparent and mitotic activity may be high, mimicking other high-grade sarcomas, carcinoma or melanoma
• Haemorrhage and blood-filled cavities

Question 6

Angiosarcoma positive stains

Answer 6

CD31
CD34
VEGF
Factor VIII

Negative: HHV8

c-MYC crucial in distinguishing angiosarcoma (positive) from mimickers (negative)

Question 7

Management of angiosarcoma

Answer 7

* Staging – MRI with gadolinium, biopsy, consider PET/CT (can metastasise to LN and lungs)

* Surgical excision with wide margins – standard excision or MMS

• Recurrence rate and metastatic disease rate is still high even with negative margins

* Systemic
o Thalidomide
o Paclitaxel
o Propanolol + vinblastine-based chemotherapy

* Radiotherapy – palliative but does not improve survival