Angiosarcoma

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Angiosarcoma

• Linda Verkruyse MD
• BMT Grand Rounds
• 10/3/03

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Patient

• 74 y/o female presented with lesion on her nose
• Biopsy positive for angiosarcoma
• Treatment
• Surgical resection with reconstruction
• Radiation
• Biopsy proven recurrence under eyebrow 1 year
  after completion of therapy

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Angiosarcoma

• Uncommon tumor
• Represents 2 of all sarcomas
• Arise from vascular endothelium or lymphatics
• Aggressive
• 5 year survival rate 10-35
• Median survival 18 months
• High rate of local recurrence after treatment
• Most patients die of metastatic disease

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Angiosarcoma

• Can occur anywhere (60 cutaneous)
• Head and neck (50)
• Extremities
• Breast
• Liver
• Heart
• Lung
• Bone
• Adrenals
• Kidney

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• Clinical Appearance (cutaneous)
• Bruise
• Red or purple plaque or nodule
• Ulcer
• Spontaneous bleeding
• Rapidly enlarging

• Diagnosis usually delayed
• Variable appearance
• Asymptomatic
• Rare low index of suspicion

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Risk Factors

• Prior radiation treatment
• Chronic lymphedema/edema (s/p breast cancer
  treatment, venous stasis)
• Vinyl chloride exposure (liver)
• Thorium dioxide (thorotrast)
• Arsenic

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Histology

• Arise from blood or lymphatic vessels
• Poorly differentiated vascular channels and
  atypical endothelial cells
• Reactive to CD31, factor 8 related antigen, and
  CD34

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Treatment

• Surgical excision
• Distant microscopic extension a problem
• Wide local excision
• Reconstruction often needed
• Radiation
• Not curative
• Combination of surgery radiation gives longer
  disease free survival

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Chemotherapy

• Standard regiments doxorubicin/ifosfamide based
• Cyclophosphamide/cisplatinum
• Doxil

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Chemotherapy

• Similarity between angiosarcoma and Kaposis
  sarcoma?
• Conflicting results investigating the presence of
  HHV-8 DNA in angiosarcoma tumors
• Some recent therapies reported based on
  treatments that have shown efficacy in KS

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Chemotherapy

• Taxol
• Study evaluated 9 patients retrospectively
• 8 patients with angiosarcoma of the scalp and 1
  of the face
• Variable treatment schedules
• 4 CR, 4 PR, 1 SD
• (Fata, F., OReilly, E., et al., Cancer 86(10),
  1999, 2034-2037)

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Chemotherapy

• Doxil interferon-alpha
• Case report of some activity
• 20mg/m2 Doxil q 2weeks 4.5 million IU
  interferon 3X/week
• Partial response lasted 11 months
• (Jackel, A., Deichmann, M., et al., British J. of
  Dermatology, 140(6), 1999, 1187-1188)

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Chemotherapy

• Interferon alpha-2a 13-cis-retinoic acid
• Case report of activity
• Interferon-alpha 2a 6 million IU/day
  13-cis-retinoic acid 1mg/kg/day
• Partial response ? stable for 20 months
• (Konstanze, S., Gille, J., et al., Archives of
  Dermatology, 135(9), 1999, 1035-1037)

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Chemotherapy

• Angiogenesis inhibitors
• Thalidomide
• Case report of some activity
• 150mg TID
• PR lasted 2 months
• (Ventura, G., Roberts, S., ASCO Abstract 2268,
  2000)
• Avastin (?)

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Our Patient

• Treatment
• Doxil (decrease in size of cervical lymph node)
• Doxil interferon-alpha
• Taxotere Bexarotene
• Taxol