Angiosarcoma

WikiDoc Resources for Angiosarcoma
Articles
Most recent articles on Angiosarcoma Most cited articles on Angiosarcoma Review articles on Angiosarcoma Articles on Angiosarcoma in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Angiosarcoma Images of Angiosarcoma Photos of Angiosarcoma Podcasts & MP3s on Angiosarcoma Videos on Angiosarcoma
Evidence Based Medicine
Cochrane Collaboration on Angiosarcoma Bandolier on Angiosarcoma TRIP on Angiosarcoma
Clinical Trials
Ongoing Trials on Angiosarcoma at Clinical Trials.gov Trial results on Angiosarcoma Clinical Trials on Angiosarcoma at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Angiosarcoma NICE Guidance on Angiosarcoma NHS PRODIGY Guidance FDA on Angiosarcoma CDC on Angiosarcoma
Books
Books on Angiosarcoma
News
Angiosarcoma in the news Be alerted to news on Angiosarcoma News trends on Angiosarcoma
Commentary
Blogs on Angiosarcoma
Definitions
Definitions of Angiosarcoma
Patient Resources / Community
Patient resources on Angiosarcoma Discussion groups on Angiosarcoma Patient Handouts on Angiosarcoma Directions to Hospitals Treating Angiosarcoma Risk calculators and risk factors for Angiosarcoma
Healthcare Provider Resources
Symptoms of Angiosarcoma Causes & Risk Factors for Angiosarcoma Diagnostic studies for Angiosarcoma Treatment of Angiosarcoma
Continuing Medical Education (CME)
CME Programs on Angiosarcoma
International
Angiosarcoma en Espanol Angiosarcoma en Francais
Business
Angiosarcoma in the Marketplace Patents on Angiosarcoma
Experimental / Informatics
List of terms related to Angiosarcoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Hemangiosarcoma; Pulmonary angiosarcoma; Vascular sarcoma

Overview

Angiosarcoma is a rare malignant vascular neoplasm of endothelial-type cells that line vessel walls. The pathogenesis of angiosarcoma is characterized by a rapid and extensively infiltrating overgrowth of vascular epithelial cells.

Historical Perspective

• Angiosarcoma was first discovered by Rosai and colleagues, in 1976.

Classification

• Angiosarcoma may be classified according to staging into 4 subtypes:

Pathophysiology

• The pathogenesis of angiosarcoma is characterized by a rapid and extensively infiltrating overgrowth of vascular epithelial cells.
• Angiosarcoma is a locally aggressive tumor with a high rate of lymph node infiltration and metastases.
• Common angiosarcoma locations, include: kidney, liver, lung, breast, and liver.
• The PTPRB /PLCG1 genes have been associated with the development of angiosarcoma, as a result generate aberrant angiogenesis.
• On gross pathology, characteristic findings of angiosarcoma, may include:

• Red/dark tan lesion
• Typically poorly circumscribed

• On microscopic histopathological analysis, characteristic findings of angiosarcoma, may include:

• Spindle cell lesion.
• Occasionally an epitheloid lesion
• Very many small capillaries of irregular shape
• Pleomorphic nuclei
• May have hobnail morphology
• Usually "red" at low power (key feature)
• Mitoses
• Cytoplasmic vacuoles
• Cells trying to form lumina

Causes

• Common causes of angiosarcoma may include:

Differentiating Angiosarcoma from Other Diseases

• Angiosarcoma must be differentiated from other diseases that cause a highly vascular mass, such as:

• Atypical vascular lesions
• Hemangioma
• Glomangiosarcoma
• Carotid body tumor
• Malignant fibrous histiocytoma of soft tissue

Epidemiology and Demographics

• Angiosarcoma is very uncommon.

Age

• Angiosarcoma is more commonly observed among patients aged [age range] years old.
• Angiosarcoma is more commonly observed among middle aged adults.

Gender

• Males are more commonly affected with angiosarcoma than females.
• The male to female ratio is 2:1.

Race

• There is no racial predilection for angiosarcoma.

Risk Factors

• Common risk factors in the development of angiosarcoma, include:

• Chronic lymphedema
• Polyvinyl chloride (PVC)
• Radiation
• Sun exposure
• Thorotrast

Natural History, Complications and Prognosis

• The majority of patients with angiosarcoma remain asymptomatic for years.
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, the majority of patients with angiosarcoma may progress to develop metastases.
• Common complications of angiosarcoma include [
• Prognosis is generally poor and the [1/5/10­year mortality/survival rate] of patients with angiosarcoma is approximately [#%].

Diagnosis

Symptoms

• Angiosarcoma is usually asymptomatic and found incidentally.
• There are no remarkable symptoms for angiosarcoma.

Physical Examination

• Patients with angiosarcoma usually appear cachectic, or normal.
• There are no remarkable physical examination findings for patients with angiosarcoma. .

Laboratory Findings

• There are no specific laboratory findings associated with angiosarcoma.

Imaging Findings

• There are no [imaging study] findings associated with angiosarcoma.

• [Imaging study 1] is the imaging modality of choice for angiosarcoma.
• On [imaging study 1], angiosarcoma is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

• Angiosarcoma may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• The mainstay of therapy for angiosarcoma is a doxorubicin-based regimen.
• For angiosarcoma, doxorubicin monotherapy is indicated as first-line therapy
• For patients with pulmonary angiosarcoma, combination of radiotherapy and immunotherapy with recombinant interleukin-2 is the treatment of choice.
• Common complications of doxorubicin, include:

• Chronic cardiotoxicity
• Mucositis
• Alopecia
• Nausea
• Vomiting

Surgery

• Surgery is the mainstay of therapy for angiosarcoma.
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of angiosarcoma.
• [Surgical procedure] can only be performed for patients with [disease stage] angiosarcoma.

Prevention

• There are no primary preventive measures available for angiosarcoma.

• Effective measures for the primary prevention of angiosarcoma include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with angiosarcoma are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].