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

Overview

Lymphangiosarcoma was first discovered by Lowenstein, in 1906, following severe posttraumatic lymphedema of arm for 5 years. 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. Lymphangiosarcoma may be caused by classical Halstedian radical mastectomy. Lymphangiosarcoma must be differentiated from other diseases that cause swelling of limb such as acquired angioedema due to C1 inhibitor deficiency, angioendotheliomatosis, angiolymphoid hyperplasia with eosinophilia, cutaneous melanoma, hereditary angioedema, lymphangiectasia, lymphangioma, and lymphocytoma cutis. On gross pathology, pachydermatous skin, edema, and reddish blue macules or nodules are characteristic findings of lymphangiosarcoma. On microscopic histopathological analysis, proliferating vascular channels, which dissect the dermal collagen, hyperchromatism, pleomorphism, mitoses, pinocytosis, intercellular junctions, cytoplasmic intermediate filaments, Weibel-Palade bodies, and erythrophagocytosis are charecteristic findings of lymphangiosarcoma. The prevalence of Stewart-Treves syndrome is approximately 400 worldwide. Common risk factors in the development of lymphangiosarcoma are lymphatic blockage, radiotherapy, mastectomy, cardiovascular diseases, and hypertension.[1]The sarcoma first appears as a bruise mark, a purplish discolorization or a tender skin nodule in the extremity, typically on the anterior surface. Lymphangiosarcoma progresses to an ulcer with crusting, and finally to an extensive necrosis involving the skin and subcutaneous tissue. Lymphangiosarcoma metastasizes quickly. Findings on biopsy and ultrastructural histologic studies include proliferating vascular channels, which dissect the dermal collagen and, often, the obliterate appendages, hyperchromatism, pleomorphism, mitoses, pinocytosis, intercellular junctions, cytoplasmic intermediate filaments, Weibel-Palade bodies, and erythrophagocytosis. Amputation of the affected limb is the most common approach to the treatment of lymphangiosarcoma.

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.[2]

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.[3]
  • 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.[4][5]
  • On gross pathology, pachydermatous skin, edema, and reddish blue macules or nodules are characteristic findings of lymphangiosarcoma
  • On microscopic histopathological analysis, proliferating vascular channels, which dissect the dermal collagen and, often, the obliterate appendages, hyperchromatism, pleomorphism, mitoses, pinocytosis, intercellular junctions, cytoplasmic intermediate filaments, Weibel-Palade bodies, and erythrophagocytosis are charectoristic findings of lymphangiosarcoma.

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 lymphangiosarcoma are lymphatic blockage, radiotherapy, mastectomy, cardiovascular diseases, and hypertension.[1]

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.
  • Prognosis is generally poor, and the 5 year survival rate of patients with lymphangiosarcomais approximately less than 5%.

Diagnosis

Symptoms

  • Symptoms of lymphangiosarcoma may include the following:
  • Bruise mark, a purplish discoloration
  • Tender skin nodule in the extremity

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 lymphangiosarcoma.

Imaging Findings

  • MRI is the imaging modality of choice to asses the local extend of lymphangiosarcoma.
  • On MRI, lymphangiosarcoma is characterized by low signal intensity on T2-weighting.
  • Chest radiography may demonstrate pulmonary metastasis.

Other Diagnostic Studies

  • Lymphangiosarcoma may also be diagnosed by measuring antibodies against factor VIII–related antigen, CD34 antigen, antikeratin antibodies, and positive staining for laminin, CD31, collagen IV, and vimentin.
  • Findings on biopsy and ultrastructural histologic studies include proliferating vascular channels which dissect the dermal collagen, hyperchromatism, pleomorphism, mitoses, pinocytosis, intercellular junctions, cytoplasmic intermediate filaments, Weibel-Palade bodies, and erythrophagocytosis.

Treatment

Medical Therapy

Surgery

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

Prevention

  • Monitoring patients with lymphedema is the primary preventive measure available for lymphangiosarcoma.

References

  1. 1.0 1.1 Sepah YJ, Umer M, Qureshi A, Khan S (2009). "Lymphangiosarcoma of the arm presenting with lymphedema in a woman 16 years after mastectomy: a case report.". Cases J. 2: 6887. PMC 2769324Freely accessible. PMID 19918554. doi:10.4076/1757-1626-2-6887. 
  2. McKeown DG, Boland PJ (2013). "Stewart-Treves syndrome: a case report.". Ann R Coll Surg Engl. 95 (5): e80–2. PMC 4165172Freely accessible. PMID 23838488. doi:10.1308/003588413X13629960046110. 
  3. Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: a report of six cases in elephantiasis chirurgica. Cancer 1948;1:64–81.
  4. Chopra, S, Ors, F, Bergin, D MRI of angiosarcoma associated with chronic lymphoedema: Stewart Treves syndrome Br J Radiol 2007 80: e310–313
  5. Schreiber H, Barry FM, Russell WC, Macon IV WL, Ponsky JL, Pories WJ. Stewart–Treves Syndrome: a lethal complication of postmastectomy lymphedema and regional immune deficiency. Arch Surgery 1979;114:82–5.

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