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Epithelioid Angiosarcoma: Symptoms and Treatment

  • Endothelial cells are the source of the malignant vascular tumor known as angiosarcoma (AS). Radiation therapy and chronic lymphedema are linked in some instances, while the exact etiology is unknown.
  • Early lesions may be mistaken for a bruise or a benign vascular disorder, making the diagnosis of AS difficult to make. Any older patient who has a history of radiation therapy and/or chronic lymphedema and a new, vascular-looking lesion should have AS suspected.
  • Excision with wide margins during surgery is the preferred course of action.

skin angiosarcoma


Symptoms of Cutaneous Angiosarcoma

Anywhere, including organ systems other than the skin, can develop angiosarcoma.

    Clinical Situations

Angiosarcoma of the skin commonly manifests in one of three clinical scenarios:

  • Geriatric patients are most likely to have scale-face AS;
  • Prior radiation therapy is a setting where radiation-associated AS can occur;
  • Stewart-Treves syndrome (KS) associated with lymphedema manifests on the extremities of people with persistent lymphedema, such as in the arm following axillary lymph node dissection for breast cancer.

    Epithelioid Angiosarcoma Symptoms

  • A macule or patch that resembles a bruise and is red or purple at first but eventually becomes larger is the early AS lesion. There is frequently an irregular border that blends into the nearby normal skin.
  • Red, purple, or even skin-colored papules, nodules, and tumors may appear as the illness gets worse.
  • AS lesions that have progressed typically bleed or ulcerate.

Diagnosis

  •  A biopsy is required to confirm the diagnosis of angiosarcoma. The usual recommendation is for a big, deep punch or incisional biopsy that includes subcutaneous fat. 
  • It must be remembered that because the vascular alterations in the shallow dermis may be mild, AS may be challenging or impossible to diagnose on superficial biopsies. A shave biopsy shouldn't be done if AS is a significant clinical concern.

Treatment

  • Treatment of SA may be:

    Drug Therapy

  • Doxorubicin-based chemotherapy has been used sporadically with mixed results.

    Treatment Methods

Surgery

  • Excision with wide margins is the preferred course of treatment when surgery is an option. The margins of angiosarcoma are notoriously difficult to determine, both clinically and histologically. 
  • In the treatment of AS, microscopically controlled surgery, or Mohs surgery, is not used.

Radiotherapy

  • Radiation therapy is typically used for big lesions that are not amenable to surgery or for recurrence following surgery. 
  • Additionally, radiation therapy may be used with surgery.


Prognosis

  • Angiosarcomas are aggressive soft-tissue tumors with 5-year survival rates ranging from 12% to 33%, according to several series. 
  • Most patients pass away between 9 and 12 months after their diagnosis.

Sources


Jeldia Website

Https://jeldia.com


American Cancer Society. (2021, November 10). Angiosarcoma. Retrieved from https://www.cancer.org/cancer/angiosarcoma.html


Memorial Sloan Kettering Cancer Center. (2021, December 7). Cutaneous Angiosarcoma. Retrieved from https://www.mskcc.org/cancer-care/types/cutaneous-angiosarcoma


National Cancer Institute. (2021, November 19). Angiosarcoma Treatment (Adult). Retrieved from https://www.cancer.gov/types/skin/patient/angiosarcoma-treatment-pdq


Mayo Clinic. (2021, October 28). Angiosarcoma. Retrieved from https://www.mayoclinic.org/diseases-conditions/angiosarcoma/symptoms-causes/syc-20351133


American Society of Clinical Oncology. (2021, November 1). Angiosarcoma. Retrieved from https://www.cancer.net/cancer-types/angiosarcoma


British Association of Dermatologists. (2021, September 15). Angiosarcoma. Retrieved from https://www.bad.org.uk/patient-information-leaflets/angiosarcoma/


Skin Cancer Foundation. (2021, November 22). Angiosarcoma. Retrieved from https://www.skincancer.org/skin-cancer-information/angiosarcoma


Cancer Research UK. (2021, December 2). Angiosarcoma. Retrieved from https://www.cancerresearchuk.org/about-cancer/angiosarcoma

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