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=== Incidence ===
=== Incidence ===
* [[Incidence]] of lymphangiosarcoma in white American women is estimated to be 1.6 per 100,000.
* [[Incidence]] of lymphangiosarcoma in white American women is estimated to be 1.6 per 100,000.<ref name="pmid8000998">{{cite journal |vauthors=Mack TM |title=Sarcomas and other malignancies of soft tissue, retroperitoneum, peritoneum, pleura, heart, mediastinum, and spleen |journal=Cancer |volume=75 |issue=1 Suppl |pages=211–44 |date=January 1995 |pmid=8000998 |doi= |url=}}</ref>


===Age===
===Age===

Revision as of 23:06, 6 January 2019

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

Synonyms and keywords: Stewart-Treves syndrome

Overview

Lymphangiosarcoma was first discovered by Lowenstein, in 1906. The index case of lymphangiosarcoma was found in a patient suffering from severe post-traumatic lymphedema of arm. 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 must be differentiated from other diseases that cause swelling of limb. The prevalence of Stewart-Treves syndrome is approximately 400 per 100,000 individuals worldwide. Common risk factors that may lead to the development of lymphangiosarcoma include lymphatic blockage, radiotherapy, mastectomy, cardiovascular diseases, and hypertension. The sarcoma first appears as a bruise mark, a purplish discoloration or a tender skin nodule in the extremity, typically on the anterior surface. Findings on biopsy and ultrastructural histologic studies suggestive of lymphangiosarcoma include proliferating vascular channels, 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. The index case of lymphangiosarcoma was found in a patient suffering from severe post-traumatic lymphedema of arm.
  • In 1948, Fred Stewart and Norman Treves first identified postmastectomy lymphedema as a precursor condition leading to lymphangiosarcoma.
  • In 1960, the first homograft skin transplantation was developed to treat lymphangiosarcoma.
  • In 1979, the concept of local immunodeficiency was first identified as a possible mechanism leading to the development of lymphangiosarcoma by Schreiber.[1]

Pathophysiology

Causes

Differentiating Lymphangiosarcoma from other Diseases

  • Lymphangiosarcoma must be differentiated from other diseases that cause swelling of limb such as[14][15][16]:

The following table differentiates various conditions that may lead to limb swelling:

Diseases Etiology Congenital Acquired Demography Clinical manifestations Lab findings Gold standard diagnosis Associated findings
Symptoms Signs CBC LFT ESR/CRP Histopathology
Appearance Fever Bleeding BP Hepatosplenomegaly Lymphadenopathy Other WBC Hb Plt
Bacillary angiomatosis [17] + Any age, usually between 20 -50 years Solitary or multiple red, purple, flesh-colored, or colorless papules ± ± Nl Nl Nl Nl Nl Nl Clinical manifestation
Arteriovenous malformation [18] + Any age Nl + Nl Nl Nl Nl Nl Nl NA Imaging
Acroangiodermatitis[19] Any age, more in males Purplish-blue to brown papules and plaques Nl
  • Paralysed legs
Nl Nl Nl Nl Nl Clinical manifesttations
Angiosarcoma [20] Adults, more in males Enlarging bruise, a blue-black nodule, or an unhealed ulceration Nl Nl Nl Nl Biopsy NA
Diseases Etiology Congenital Acquired Demography Appearance Fever Bleeding BP Hepatosplenomegaly Lymphadenopathy Other WBC Hb Plt LFT ESR/CRP Histopathology Gold standard diagnosis Associated findings
Masson's hemangioma [21] Rare
  • Normal
Nl Nl Nl Nl Nl Nl Biopsy
Seborrheic keratosis [22] + Any age Nl Nl Nl Nl Nl Nl
  • Papillomatous epithelial proliferation containing horn cysts
Clinical manifestations
Systemic lupus erythematosus (SLE) [23] More common in female, typically in the 20 to 30 years ± ± ± Nl Nl Clinical manifestations
Pyogenic granuloma [24]
  • Trauma
  • Hormonal influences
  • Viruses
  • Cytogenetic clonal deletion abnormalities
+ + Any age, usually in 20-30 years + Nl Nl Nl Nl Nl Nl Clinical manifestation NA
Benign lymphangioendothelioma [25] + Any ages, median age is 50 years Nl Nl Nl Nl Nl Nl
  • Thin-walled endothelial-lined spaces that are interspersed between strands of collagen
Biopsy NA
Cavernous hemangioma [26] Usually in third to fifth decades of life.
  • Painless, slowly progressive protrusion or bulging of their globe
Nl Nl Nl Nl Nl Nl
  • Engorged vascular channels, which are tightly knit and separated by fibrous septae
Clinical manidestation
Diseases Etiology Congenital Acquired Demography Appearance Fever Bleeding BP Hepatosplenomegaly Lymphadenopathy Other WBC Hb Plt LFT ESR/CRP Histopathology Gold standard diagnosis Associated findings

Epidemiology and Demographics

Prevalence

  • Lymphangiosarcoma is a rare entity and 300 cases of lymphangiosarcoma after breast cancer have been reported worldwide.[27][28]

Incidence

  • Incidence of lymphangiosarcoma in white American women is estimated to be 1.6 per 100,000.[29]

Age

  • Lymphangiosarcoma is more commonly observed among middle-aged or elderly (mostly between sixth and seventh decade of life which correlates with a higher incidence of breast cancer.[30]

Gender

  • Female are more commonly affected with lymphangiosarcoma than male.[31]

Race

  • There is no racial predilection for lymphangiosarcoma.

Risk Factors

Common risk factors in the development of lymphangiosarcoma are[32][28][33]:

Natural History, Complications and Prognosis

Diagnosis

Symptoms

Symptoms of lymphangiosarcoma may include the following[36]:

Physical Examination

Physical examination may be remarkable for[37][38][39]:

  • Lymphedema- Nontender and non pitting edema of the affected area.
  • Lymphangiosarcoma-
    • 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

Imaging Findings

  • MRI with intravenous contrast is the imaging modality of choice to asses the local extend of lymphangiosarcoma.
  • On MRI, lymphangiosarcoma is characterized by a soft tissue mass with extension through the sub cutaneous tissue and up to the muscle layer with enhancement.
  • Chest radiography and chest CT scan 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, 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[40].

Prevention

  • Treatment of lymphedema is the primary preventive measure available for lymphangiosarcoma.

References

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  2. Sun S, Chen S, Liu F, Wu H, McHugh J, Bergin IL; et al. (2015). "Constitutive Activation of mTORC1 in Endothelial Cells Leads to the Development and Progression of Lymphangiosarcoma through VEGF Autocrine Signaling". Cancer Cell. 28 (6): 758–772. doi:10.1016/j.ccell.2015.10.004. PMC 4828306. PMID 26777415.
  3. Mackenzie DH (1971). "Lymphangiosarcoma arising in chronic congenital and idiopathic lymphoedema". J Clin Pathol. 24 (6): 524–9. PMC 477086. PMID 5094684.
  4. Acharya AS, Sulhyan K, Ramteke R, Kunghadkar V (2013). "Cutaneous lymphangiosarcoma following chronic lymphedema of filarial origin". Indian J Dermatol. 58 (1): 68–70. doi:10.4103/0019-5154.105314. PMC 3555379. PMID 23372218.
  5. Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: a report of six cases in elephantiasis chirurgica. Cancer 1948;1:64–81.
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  7. LISZAUER S, ROSS RC (1957). "Lymphangiosarcoma in lymphoedema". Can Med Assoc J. 76 (6): 475–7. PMC 1823629. PMID 13413767.
  8. 8.0 8.1 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. doi:10.4076/1757-1626-2-6887. PMC 2769324. PMID 19918554.
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  17. Tappero JW, Perkins BA, Wenger JD, Berger TG (July 1995). "Cutaneous manifestations of opportunistic infections in patients infected with human immunodeficiency virus". Clin. Microbiol. Rev. 8 (3): 440–50. PMC 174635. PMID 7553576.
  18. Whitehead KJ, Smith MC, Li DY (February 2013). "Arteriovenous malformations and other vascular malformation syndromes". Cold Spring Harb Perspect Med. 3 (2): a006635. doi:10.1101/cshperspect.a006635. PMC 3552339. PMID 23125071.
  19. Lugović L, Pusić J, Situm M, Buljan M, Bulat V, Sebetić K, Soldo-Belić A (2007). "Acroangiodermatitis (pseudo-Kaposi sarcoma): three case reports". Acta Dermatovenerol Croat. 15 (3): 152–7. PMID 17868541.
  20. Barttelbort SW, Stahl R, Ariyan S (July 1989). "Cutaneous angiosarcoma of the face and scalp". Plast. Reconstr. Surg. 84 (1): 55–9. PMID 2734404.
  21. Park KK, Won YS, Yang JY, Choi CS, Han KY (July 2012). "Intravascular Papillary Endothelial Hyperplasia (Masson tumor) of the Skull : Case Report and Literature Review". J Korean Neurosurg Soc. 52 (1): 52–4. doi:10.3340/jkns.2012.52.1.52. PMC 3440504. PMID 22993679.
  22. Noiles K, Vender R (2008). "Are all seborrheic keratoses benign? Review of the typical lesion and its variants". J Cutan Med Surg. 12 (5): 203–10. doi:10.2310/7750.2008.07096. PMID 18845088.
  23. Uva L, Miguel D, Pinheiro C, Freitas JP, Marques Gomes M, Filipe P (2012). "Cutaneous manifestations of systemic lupus erythematosus". Autoimmune Dis. 2012: 834291. doi:10.1155/2012/834291. PMC 3410306. PMID 22888407.
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  25. Guillou L, Fletcher CD (August 2000). "Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series". Am. J. Surg. Pathol. 24 (8): 1047–57. PMID 10935645.
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  27. Sepah YJ, Umer M, Qureshi A, Khan S (September 2009). "Lymphangiosarcoma of the arm presenting with lymphedema in a woman 16 years after mastectomy: a case report". Cases J. 2: 6887. doi:10.4076/1757-1626-2-6887. PMID 19918554.
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  29. Mack TM (January 1995). "Sarcomas and other malignancies of soft tissue, retroperitoneum, peritoneum, pleura, heart, mediastinum, and spleen". Cancer. 75 (1 Suppl): 211–44. PMID 8000998.
  30. Chung KC, Kim HJ, Jeffers LL (November 2000). "Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients". J Hand Surg Am. 25 (6): 1163–8. doi:10.1053/jhsu.2000.18490. PMID 11119680.
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  40. 40.0 40.1 40.2 Stanczyk M, Gewartowska M (2014). "Stewart-Treves syndrome". Indian J Med Res. 139 (1): 179. PMC 3994737. PMID 24604055.