Lymphangiosarcoma

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

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

Synonyms and keywords: Synonym 1; Synonym 2; Synonym 3

Historical Perspective

• Lymphangiosarcoma was first discovered by Lowenstein, in 1906, following severe posttraumatic lymphedema of arm for 5 years
• In 1948, postmastectomy lymphedema were first identified in the pathogenesis of lymphangiosarcoma by Fred Stewart and Norman Treves.
• In 1960, the first homograft skin transplantation was developed by 1960 to treat diagnose lymphangiosarcoma.
• In 1979, the concept of local immunodeficiency was first identified in the pathogenesis of lymphangiosarcoma by Schreiber.

Classification

• [Disease name] may be classified according to [classification method] into [number] subtypes/groups:

• [group1]
• [group2]
• [group3]

• Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

• Lymphangiosarcoma is a rare malignant tumor which occurs in long-standing cases of Primary or Secondary Lymphedema. It involves either the upper or lower lymphedemateous extremities but is most common in upper extremities.
• When it occurs following mastectomy it is known as Stewart-Treves Syndrome.
• Stewart and Treves, proposed that a cancer causing agent is present in lymphedematous limbs.
• Schreiber et al. proposed that local immunodeficiency as a result of lymphedema results in a "immunologically privileged site" in which the sarcoma is able to develop.
• The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
• On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
• On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Causes

• Lymphangiosarcoma may be caused by classical Halstedian radical mastectomy
• [Disease name] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
• There are no established causes for [disease name].

Differentiating [disease name] from other Diseases

• [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics

• The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
• In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

• Patients of all age groups may develop [disease name].
• [Disease name] is more commonly observed among patients aged [age range] years old.
• [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

• [Disease name] affects men and women equally.
• [Gender 1] are more commonly affected with [disease name] than [gender 2].
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

• There is no racial predilection for [disease name].
• [Disease name] usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop [disease name].

Risk Factors

• Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The sarcoma first appears as a bruise mark, a purplish discolorization or a tender skin nodule in the extremity, typically on the anterior surface. It progresses to an ulcer with crusting, and finally to an extensive necrosis involving the skin and subcutaneous tissue. It metastasizes quickly.
• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] 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 [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• [Disease name] is usually asymptomatic.
• Symptoms of [disease name] may include the following:

• [symptom 1]
• [symptom 2]
• [symptom 3]
• [symptom 4]
• [symptom 5]
• [symptom 6]

Physical Examination

• Physical examination may be remarkable for:

• Bruise mark
• A purplish discolorization
• Tender skin nodule in the extremity, typically on the anterior surface
• Ulcer with crusting
• Extensive necrosis involving the skin and subcutaneous tissue

Laboratory Findings

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

Imaging Findings

• There are no [imaging study] findings associated with [disease name].
• [Imaging study 1] is the imaging modality of choice for [disease name].
• On [imaging study 1], [disease name] 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

• [Disease name] 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 [disease name]; the mainstay of therapy is supportive care.
• The mainstay of therapy for [disease name] 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

• Amputation of the affected limb is the most common approach to the treatment of lymphangiosarcoma.

Prevention

• There are no primary preventive measures available for [disease name].
• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
• Once diagnosed and successfully treated, patients with [disease name] are followedup every [duration]. Followup testing includes [test 1], [test 2], and [test 3].