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: Stewart-Treves syndrome

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.

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

Differentiating Lymphangiosarcoma from other Diseases

• Lymphangiosarcoma must be differentiated from other diseases that cause swellling of limb such as:

• Acquired Angioedema Due to C1 Inhibitor Deficiency
• Angioendotheliomatosis
• Angiolymphoid Hyperplasia With Eosinophilia
• Cutaneous Melanoma
• Hereditary Angioedema
• Lymphangiectasia
• Lymphangioma
• Lymphocytoma Cutis

Epidemiology and Demographics

• The prevalence of Stewart-Treves syndrome is approximately 400 worldwide.
• In 1962, the incidence of Stewart-Treves syndrome was estimated to be 0.45% in patients who survive at least 5 years after radical mastectomy and 0.03% of patients surviving 10 or more years after radical mastectomy.

Age

• Lymphangiosarcoma is more commonly observed among middle-aged or elderly.

Gender

• Female are more commonly affected with lymphangiosarcoma than male.

Race

• There is no racial predilection for lymphangiosarcoma.

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].