Angiosarcoma

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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.

Historical Perspective

  • Angiosarcoma was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].

Classification

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

Pathophysiology

  • The pathogenesis of angiosarcoma is characterized by an overgrowth of vascular epithelial cells.
  • Common angiosarcoma locations, include: kidney, liver, lung, breast, and liver.
  • The [gene name] gene/Mutation in [gene name] has been associated with the development of angiosarcoma, involving the [molecular pathway] pathway.
  • 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 [duration/years].
  • Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
  • If left untreated, [#%] of patients with angiosarcoma may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of angiosarcoma include [complication 1], [complication 2], and [complication 3].
  • Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with angiosarcoma is approximately [#%].

Diagnosis

Diagnostic Criteria

  • The diagnosis of angiosarcoma is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • Angiosarcoma is usually asymptomatic.
  • Symptoms of angiosarcoma may include the following:
  • [symptom 1]
  • [symptom 2]
  • [symptom 3]
  • [symptom 4]
  • [symptom 5]
  • [symptom 6]

Physical Examination

  • Patients with angiosarcoma usually appear [general appearance].
  • Physical examination may be remarkable for:
  • [finding 1]
  • [finding 2]
  • [finding 3]
  • [finding 4]
  • [finding 5]
  • [finding 6]

Laboratory Findings

  • There are no specific laboratory findings associated with angiosarcoma.
  • A [positive/negative] [test name] is diagnostic of angiosarcoma.
  • An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of angiosarcoma.
  • Other laboratory findings consistent with the diagnosis of angiosarcoma include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

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

  • There is no treatment for angiosarcoma; the mainstay of therapy is supportive care.
  • The mainstay of therapy for angiosarcoma is [medical therapy 1] and [medical therapy 2].
  • [Medical therapy 1] acts by [mechanism of action1].
  • Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

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].

References