Angiosarcoma

Angiosarcoma 

Summary

4 Interesting Facts of Angiosarcoma

  1. Angiosarcoma (AS) is a malignant vascular tumor derived from endothelial cells. Although its cause is not fully understood, chronic lymphedema and radiation therapy are implicated in some cases.
  2. The diagnosis of AS requires a high index of suspicion, and early lesions may be misinterpreted as a bruise or benign vascular condition. AS should be suspected in any older patient, particularly someone with a history of radiation and/or chronic lymphedema and a new, vascular-appearing lesion.
  3. If surgery is possible, excision with generous margins is the treatment of choice.
  4. Angiosarcomas are aggressive soft-tissue tumors, with multiple series reporting a 5-year survival rate of 12% to 33%. Most persons with AS die within 9 to 12 months of diagnosis.

Workup

Physical Examination

  • Angiosarcoma (AS) may occur anywhere, including organ systems other than the skin. Cutaneous angiosarcoma typically presents in one of three clinical situations:
    Scalp-face AS is most common in geriatric patients.
  • Radiation-associated AS occurs in the field of prior radiation therapy.
  • Lymphedema-associated KS (Stewart-Treves syndrome) occurs on the extremities of persons with chronic lymphedema, such as that occurring in the arm after axillary lymph node dissection for breast carcinoma.
  • The earliest lesion of AS appears as a red or purple bruise-like macule or patch that progressively enlarges. There is often an ill-defined margin that merges with adjacent normal skin.
  • As the condition worsens, red, purple, or even skin-colored papules, nodules, and tumors may develop.
  • Advanced lesions of AS frequently bleed or ulcerate.

Diagnostic Procedures

  • The diagnosis of angiosarcoma must be established by biopsy. Usually, a large and deep punch or incisional biopsy, to include subcutaneous fat, is recommended.
  • It must be understood that AS may be difficult or impossible to diagnose on superficial biopsies because the vascular changes in the shallow dermis may be subtle. When AS is a serious clinical consideration, a shave biopsy should not be performed.

Treatment

Drug Therapy

  • Chemotherapy, primarily doxorubicin, has been used with limited success.

Treatment Procedures

  • If surgery is possible, excision with generous margins is the treatment of choice. Angiosarcoma is notoriously difficult to excise because the margins are difficult to determine, clinically and histologically. Micrographically controlled surgery (Mohs surgery) has no role in the management of AS.
  • In large lesions that are not amenable to surgery, or in the case of recurrence after surgery, usually radiation therapy is employed. Radiation therapy may also be used in conjunction with surgery.

References

Dossett LA, et al. Cutaneous angiosarcoma. Curr Probl Cancer. 2015;39(4):258-263.Cross Reference 

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