January 14, 2007


Synonyms and related keywords: angiosarcoma, hemangioendothelioma, lymphangioendothelioma, hemangiosarcoma, hemangioblastoma, lymphangiosarcoma, angioendothelioma, malignant angioma, malignant endothelioma, malignant neoplasm, soft tissue sarcoma, soft tissue angiosarcoma, angiosarcoma of the soft tissue, cutaneous angiosarcoma, angiosarcoma of the liver, breast angiosarcoma, angiosarcoma of the breast, bone angiosarcoma, angiosarcoma of the bone, head and neck angiosarcoma, visceral angiosarcoma, hepatic angiosarcoma, lung angiosarcoma, pulmonary angiosarcoma, heart angiosarcoma, cardiac angiosarcoma


Background: Angiosarcomas are uncommon malignant neoplasms characterized by rapidly proliferating, extensively infiltrating anaplastic cells derived from blood vessels and lining irregular, blood-filled spaces. Specialists apply the term angiosarcoma to a wide range of malignant endothelial vascular neoplasms that affect a variety of sites. Angiosarcomas are aggressive and tend to recur locally, spread widely, and have a high rate of lymph node and systemic metastases. The rate of tumor-related death is high.

Pathophysiology: Angiosarcomas arising at different sites and in different organs have some distinct features. Angiosarcomas may occur in any region of the body but are more frequent in skin and soft tissue. Angiosarcomas also can originate in the liver, breast, spleen, bone, or heart.


Mortality/Morbidity: All angiosarcomas tend to be aggressive and often are multicentric. These tumors have a high local recurrence rate and metastasis because of their intrinsic biologic properties and because they often are misdiagnosed, leading to a poor prognosis and a high mortality rate. Malignant vascular tumors are clinically aggressive, difficult to treat, and have a reported 5-year survival rate of less than 20%. Advanced stage at presentation and lack of extensive excision are associated with higher recurrence, distant metastasis rates, and worsened survival.




History: Angiosarcomas are insidious, and they may not produce symptoms until the disease is well advanced. History should focus on identifying risk factors, but most patients do not have these preceding factors.

Physical: The physical examination often is unremarkable; however, subtle findings may provide clues to early detection.

Causes: The etiology of most cases of angiosarcoma is unknown. The tumors may develop as a complication of a preexisting condition. The following factors may be associated with tumor development:


Cystosarcoma Phyllodes
Hemangiomas, Hepatic
Kaposi Sarcoma
Metastatic Cancer, Unknown Primary Site

Other Problems to be Considered:

Amelanotic melanoma
Spindle-cell malignant melanoma
Pyogenic granuloma


Lab Studies:

Imaging Studies:


Histologic Findings: All angiosarcomas have similar microscopic findings, with vascular spaces more or less obvious and lined by tumor cells showing atypia. Low-grade lesions have vascular spaces lined by large plump endothelial cells that penetrate the stroma and papillary fronds of cells that project into the lumen. Higher-grade lesions are more cellular, with atypical cells and abnormal mitoses.

The main problem in the diagnosis of angiosarcoma is histopathologic recognition. Angiosarcoma may be confused with vascular tumors of intermediate malignancy (eg, epithelioid and spindle cell hemangioendotheliomas, histioid hemangioma, and malignant endovascular papillary angioendothelioma). In its more benign form, angiosarcoma may be confused with hemangiomas. In its more aggressive form, irregular sheets of anaplastic cells may have only poorly defined vascular channels and may be difficult to differentiate from anaplastic melanomas and carcinomas.

These tumors also are distinct from other malignant vasoformative tumors, including Kaposi sarcoma and malignant hemangiopericytoma. The diagnosis of angiosarcoma can be confirmed by immunohistochemical staining, described as follows:

Staging: The characteristics of the primary tumor (T), spread to regional lymph nodes (N), and the involvement by the tumor of distant lymph nodes and other distant organs and tissues (metastasis, ie, M), form the basis of the American Joint Committee on Cancer (AJCC) staging of cancer and are used widely in the United States. The T, N, and M are further divided into Tx, T0, T1, T2, T3, T4; Nx, N0, N1, N2, N3; and Mx, M0, and M1. Tx indicates that the primary tumor cannot be assessed. This indicates carcinoma in situ, and stage IV indicates metastasis.


Medical Care:

Surgical Care:


The goals of pharmacotherapy are to reduce morbidity, prevent complications, and eradicate the cancer.

Drug Category: Antineoplastic agents -- Inhibit cell growth and proliferation.
Drug Name
Doxorubicin (Adriamycin) -- A cytotoxic anthracycline antibiotic isolated from Streptomyces peucetius var. caesius. These drugs are structurally similar to tetracycline and interfere with DNA production by the cell. All cells can be affected, but rapidly producing cells are damaged. Active against many cancers and has been in use for decades. A clear orange-red powder or liquid only administered IV. Binds DNA and inhibits nucleic acid synthesis. Also a powerful iron chelator and iron-doxorubicin complex, induces production of free radicals that can destroy DNA and cancer cells.
Functional properties of a drug can be substantially affected by liposomal encapsulation. Liposomes used in different drug products can vary in their chemical and physical properties. These differences can substantially affect functional properties among liposomal drug products.
May continue treatment for as long as patient shows progress, shows no evidence of cardiotoxicity, and continues to tolerate treatment; PPE, stomatitis, or hematological toxicity may require doses to be delayed or reduced. Minimum of 4 courses recommended.
Adult DoseDoxorubicin HCL injection (Adriamycin): 60-75 mg/m2 IV as a single dose, repeat q21d; alternatively, 20-30 mg/m2 IV qd for 3 d, repeat in 4 wk; or 20 mg/m2 qwk
Doxorubicin HCl liposome injection (Doxil): 50 mg/m2 (doxorubicin HCL equivalent) IV 1 mg/min, if tolerated, increase rate of infusion to complete administration over 1 h; repeat q4wk
Pediatric Dose35-75 mg/m2 IV as a single dose q21d; or 20 mg/m2 qwk
ContraindicationsDocumented hypersensitivity; in severe CHF, if LVEF is less than 40%, therapy should not be instituted; preexisting myelosuppression; previous treatment with complete cumulative doses of other anthracyclines or anthracenediones
InteractionsDecreases digoxin plasma levels and renal excretion; allopurinol may enhance antitumor activity; toxicity increases with cyclophosphamide, mercaptopurine, and streptozocin; verapamil increases cell toxicity
Pregnancy D - Unsafe in pregnancy
PrecautionsTotal dose should not exceed 400 mg/m2 in patients with previous or concomitant treatment (eg, cyclophosphamide, daunorubicin, radiation of cardiac region), otherwise do not exceed 550 mg/m2; irreversible cardiac toxicity may occur as total dosage approaches 550 mg/m2; extravasation results in severe local tissue necrosis


Further Inpatient Care:

Further Outpatient Care:




Patient Education:


Medical/Legal Pitfalls:

Special Concerns:


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