Angiosarcoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Shyam Patel [2]; Associate Editor(s)-in-Chief: Mohsen Basiri M.D.
Synonyms and keywords: Hemangiosarcoma; Pulmonary angiosarcoma; Vascular sarcoma

Overview[edit]

Angiosarcoma is a rare malignant vascular neoplasm of endothelial-type cells that line vessel walls. The peak age of incidence appears to be the 7th decade, and men are affected more commonly than women. Angiosarcoma was first described by Dr. Juan Rosai, in 1976. The pathogenesis of angiosarcoma is characterized by a rapid and extensively infiltrating overgrowth of vascular endothelial cells. Angiosarcoma may arise in any part of the body, but is more common in soft tissues than in bones. Common angiosarcoma locations include the head and neck area, kidney, liver, lung, and and the most common site of radiation-induced angiosarcoma development is the breast. The PTPRB/PLCG1 genes are associated with the development of angiosarcoma; mutation of these genes result in aberrant angiogenesis. The imaging modality of choice for diagnosing angiosarcoma will depend on the location. For pulmonary angiosarcoma, the imaging modality of choice is CT scan with intravenous contrast. For other types angiosarcoma, the imaging modality of choice is MRI. On CT scan, findings suggestive of angiosarcoma may include vascular invasion, nodular enhancement (common), and a hypoattenuating mass. The mainstay adjuvant therapy for angiosarcoma is a doxorubicin-based regimen. The response rate for chemotherapy in patients with angiosarcoma is poor.

Historical Perspective[edit]

  • Angiosarcoma was first discovered by Dr. Juan Rosai, M.D. and colleagues in 1976.<ref name="pmid24946325">{{#invoke:Citation/CS1|citation

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Classification[edit]

  • Angiosarcoma may be classified according to the clinical heterogeneity into two main groups, and every group can be subdivided into subtypes according to the anatomical location and etiology:<ref name="pmid16053668">{{#invoke:Citation/CS1|citation

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Angiosarcoma
Primary Secondary
Cutaneous Post Radiation Angiosarcoma
Breast Lymphedema-associated Angiosarcoma
Soft tissue and Bone Angiosarcoma due to exposure to mutatgens
Visceral -

Pathophysiology[edit]

  • The pathogenesis of angiosarcoma is characterized by a rapid and extensive infiltrating overgrowth of vascular endothelial cells.<ref name="pmid20537949">{{#invoke:Citation/CS1|citation

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    • KDR mutations are seen in primary breast angiosarcoma regardless of exposure to radiation.
    • High level MYC amplification is seen is seen in radiation-induced and lymphedema-associated angiosarcoma.
    • FLT4 amplification has been detected in 25% of secondary angiosarcomas.
Image courtesy of Yale Rosen, contributed by Wikimedia commons
  • On gross pathology, characteristic findings of angiosarcoma may include red/dark tan papules or noduls, which are ytpically poorly circumscribed with central earas of necrosis and hemoorrhage.<ref name="pmid20537949">{{#invoke:Citation/CS1|citation

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  • On microscopic histopathological analysis, characteristic findings of angiosarcoma may include irregular anastomosing vascular spaces lined by [[endothelial] cells.<ref name="pmid17998731">{{#invoke:Citation/CS1|citation

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  • The tumor cells in solid area are characterized by a spindled appearance and contain Weibel-Palade bodies.<ref name="pmid28477885">{{#invoke:Citation/CS1|citation

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Causes[edit]

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Differentiating Angiosarcoma from Other Diseases[edit]

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Epidemiology and Demographics[edit]

  • The incidence of angiosarcomas can be calculated approximately 1.2 per 1,000.000 person.<ref name="pmid20537949">{{#invoke:Citation/CS1|citation

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  • Angiosarcoma is more commonly observed among patients aged between 40 to 75 years old.The peak age of incidence appears is the 7th decade,<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
  • Males are more commonly affected with angiosarcoma than females.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
  • The male to female ratio is 2:1.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
  • There is no racial predilection for angiosarcoma. however, African-Americans in the U.S are rarely affected.<ref name="pmid12778019">{{#invoke:Citation/CS1|citation

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Risk Factors[edit]

Common risk factors in the development of angiosarcoma include chronic lymphedema, chronic exposure to polyvinyl chloride (PVC), radiation exposure, and exposure to Thorotrast.<ref name="pmid20537949">{{#invoke:Citation/CS1|citation |CitationClass=journal }}</ref>

Natural History, Complications and Prognosis[edit]

  • The majority of patients with angiosarcoma remain asymptomatic for years.<ref name="pmid20537949">{{#invoke:Citation/CS1|citation

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  • Prognosis is generally poor; the 5-­year survival rate of patients with angiosarcoma is approximately 12-33%.
  • Poor prognostic factors include patient age (> 65 years), retroperitoneal location, and large tumor size.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>

Diagnosis[edit]

Diagnostic Study of Choice[edit]

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Staging[edit]

  • According to the American Joint Committee on Cancer (AJCC)/Union for International Cancer Control (UICC) and by Enneking classification, soft tissue sarcomas are classified to different stages based on the primary tumor characteristics, histological grading and the local or distant tumor involvement.
  • Table below provides summarized information regarding staging of angiosarcoma:<ref name="pmid28128848">{{#invoke:Citation/CS1|citation

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Stage Grade Site Metastasis
Ia Low grade (G1) Intracompartmental (T1) No metastasis (M0)
Ib Low grade (G1) Extracompartmental (T2) No metastasis (M0)
IIa High grade (G2) Intracompartmental (T1) No metastasis (M0)
IIb High grade (G2) Extracompartmental (T2) No metastasis (M0)
IIIa Low or High grade (G1-G2) Intracompartmental (T1) Metastasis (M1)
IIIb Low or High grade (G1-G2) Extracompartmental (T2) Metastasis (M1)

History and Symptoms[edit]

  • Angiosarcomas occur at different anatomic sites and grow insidiously, then they can present with various misleading symptoms.<ref name="YoungBrown2010">{{#invoke:Citation/CS1|citation

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  • The most common clinical manifestation is a gradually enlarging, painless mass.<ref name="pmid28471264">{{#invoke:Citation/CS1|citation

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  • Some patients complain of pain or symptoms due to compression of adjacent neurovascular structures that causes pain or edema in an extremity.
  • Secondary angiosarcomas include radiation-Induced and lymphedema-associated angiosarcoma have a distinct feature, presenting as single or several ecchymotic maculopapular cutaneous lesions in the radiation field or in areas exposed to chronic lymphedema.<ref name="pmid26617830">{{#invoke:Citation/CS1|citation

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Physical Examination[edit]

  • Patients with angiosarcoma may appear cachexic or normal. In cutaneous angiosarcoma, physical examination findings may include:
  • Primary cutaneous, head and neck and breast angiosarcoma may present with skin thickening, erythema, or bruise or skin ulceration, and blushed purple-red papule.<ref name="pmid7199829">{{#invoke:Citation/CS1|citation

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Laboratory Findings[edit]

  • There are no specific laboratory findings associated with angiosarcoma.

Electrocardiogram[edit]

Echocardiography or Ultrasound[edit]

  • On ultrasound imaging, angiosarcoma may have variable features according to the location of the tumor. It may appear as an echogenic, nodular, or lobulated mass.<ref name="BendelMaleszewski2011">{{#invoke:Citation/CS1|citation

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X-ray[edit]

  • An x-ray may be helpful in the diagnosis of bone angiosarcoma.
  • Findings on an x-ray suggestive of diagnostic include solitary lytic lesion, with irregular borders or a mixed lytic-sclerotic pattern.<ref name="pmid11201031">{{#invoke:Citation/CS1|citation

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Imaging Findings[edit]

  • The imaging modality of choice for angiosarcoma will depend on the location.
  • For pulmonary angiosarcoma, the imaging modality of choice is CT scan with intravenous contrast.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>
  • For other types angiosarcoma, the imaging modality of choice is MRI.

CT Scan[edit]

On CT, findings of angiosarcoma may include vascular invasion, nodular enhancement (common), and multicentric lesions.<ref name="angio">Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003 May. 15(3):239-52</ref>

MRI[edit]

  • On MRI, findings of angiosarcoma may include:<ref name="pmid28471264">{{#invoke:Citation/CS1|citation

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Other Imaging Findings[edit]

  • There are no other imaging findings associated with angiosarcoma.

Other Diagnostic Studies[edit]

  • There are no other diagnostic studies associated with angiosarcoma.

Treatment[edit]

Medical Therapy[edit]

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  • However, taxane-based regimen may be preferred for initial therapy.
  • Paclitaxel is effective for advanced angiosarcoma.<ref name="pmid15948172">{{#invoke:Citation/CS1|citation

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  • In addition, some vascular biologic molecules, with antiangiogenic characteristics including bevacizumab, sunitinib, and sorafenib, and with or without cytotoxic chemotherapy have shown dramatic responses in a small number of angiosarcoma patients.<ref name="pmid27274393">{{#invoke:Citation/CS1|citation

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Surgery[edit]

  • The mainstay of treatment for angiosarcoma is complete surgical resection with wide margins for local and locoregional disease in combination with preoperative or postoperative radiotherapy.<ref name="pmid7977971">{{#invoke:Citation/CS1|citation

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  • Surgical resection in combination with radiation therapy is the treatment of choice for small angiosarcomas.<ref name="pmid20537949">{{#invoke:Citation/CS1|citation

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  • Complete surgical resection with wide margins is preferred for local and locoregional angiosarcoma.<ref name="pmid20537949">{{#invoke:Citation/CS1|citation

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  • Owing to the tendency for local infiltration, surgical resection should be associated with preoperative or postoperative radiotherapy.<ref name="pmid20960566">{{#invoke:Citation/CS1|citation

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  • Surgery is not recommended on patients with large sized angiosarcomas.
  • It usually occurs after a median of six months locally or distantly and the three-year disease-free and overall survival rates both are low.<ref name="pmid23428947">{{#invoke:Citation/CS1|citation

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Primary Prevention[edit]

Secondary Prevention[edit]

References[edit]

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