Lymphangiosarcoma

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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, 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 must be differentiated from other diseases that cause swelling of limb. 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. 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. 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 was first identified in the pathogenesis of lymphangiosarcoma by Fred Stewart and Norman Treves.
  • In 1960, the first homograft skin transplantation was developed to treat 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 cases of chronic lymphedema[3].
  • Lymphedema is an abnormal collection of protein-rich fluid in the interstitium resulting from obstruction of lymphatic drainage[4].
  • Lymphatic obstruction causes an increase in the protein content of the extravascular tissue, with subsequent retention of water and swelling of the soft tissue.
  • The increase in the extravascular protein stimulates proliferation of fibroblasts, organization of the fluid, and the development of a non pitting swelling of the affected extremity.
  • Lymphangiosarcoma arises from the endothelial cells of lymphatic vessels or blood vessels[5].
  • When it occurs following mastectomy it is known as Stewart-Treves Syndrome.[6]
  • 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.
  • On gross pathology, pachydermatous skin, edema, and reddish blue macules or nodules are characteristic findings of lymphangiosarcoma[7].
  • Lymphangiosarcoma can be classified into 3 stages[8][1][9].

Causes

Differentiating Lymphangiosarcoma from other Diseases

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

Epidemiology and Demographics

  • There are 400 cases of lymphangiosarcoma reported worldwide[17].

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[18][17][19]:

Natural History, Complications and Prognosis

Diagnosis

Symptoms

Symptoms of lymphangiosarcoma may include the following[22]:

Physical Examination

Physical examination may be remarkable for[23][24][25]:

  • 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[26].

Prevention

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

References

  1. 1.0 1.1 1.2 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. 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. PMC 4828306Freely accessible. PMID 26777415. doi:10.1016/j.ccell.2015.10.004. 
  4. Mackenzie DH (1971). "Lymphangiosarcoma arising in chronic congenital and idiopathic lymphoedema.". J Clin Pathol. 24 (6): 524–9. PMC 477086Freely accessible. PMID 5094684. 
  5. Acharya AS, Sulhyan K, Ramteke R, Kunghadkar V (2013). "Cutaneous lymphangiosarcoma following chronic lymphedema of filarial origin.". Indian J Dermatol. 58 (1): 68–70. PMC 3555379Freely accessible. PMID 23372218. doi:10.4103/0019-5154.105314. 
  6. Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: a report of six cases in elephantiasis chirurgica. Cancer 1948;1:64–81.
  7. Barnett WO, Hardy JD, Hendrix JH (1969). "Lymphangiosarcoma following post-mastectomy lymphedema.". Ann Surg. 169 (6): 960–8. PMC 1387587Freely accessible. PMID 5770234. 
  8. LISZAUER S, ROSS RC (1957). "Lymphangiosarcoma in lymphoedema.". Can Med Assoc J. 76 (6): 475–7. PMC 1823629Freely accessible. PMID 13413767. 
  9. FROIO GF, KIRKLAND WG (1952). "Lymphangiosarcoma in post-mastectomy lymphedema.". Ann Surg. 135 (3): 421–5. PMC 1802333Freely accessible. PMID 14903872. 
  10. Agale SV, Khan WA, Chawlani K (2013). "Chronic lymphedema of filarial origin: a very rare etiology of cutaneous lymphangiosarcoma.". Indian J Dermatol. 58 (1): 71–3. PMC 3555380Freely accessible. PMID 23372219. doi:10.4103/0019-5154.105315. 
  11. HALL-SMITH SP, HABER H (1954). "Lymphangiosarcoma in postmastectomy lymphoedema.". Proc R Soc Med. 47 (3): 174–5. PMC 1918587Freely accessible. PMID 13155501. 
  12. MARSHALL JF (1955). "Lymphangiosarcoma of the arm following radical mastectomy.". Ann Surg. 142 (5): 871–4. PMC 1465017Freely accessible. PMID 13269040. 
  13. KETTLE JH (1957). "Lymphangiosarcoma following post-mastectomy lymphoedema.". Br Med J. 1 (5012): 193–4. PMC 1974216Freely accessible. PMID 13383227. 
  14. Pereira ES, Moraes ET, Siqueira DM, Santos MA (2015). "Stewart Treves Syndrome.". An Bras Dermatol. 90 (3 Suppl 1): 229–31. PMC 4540559Freely accessible. PMID 26312725. doi:10.1590/abd1806-4841.20153685. 
  15. RYDELL JR, JENNINGS WK, SMITH ET (1958). "Postmastectomy lymphedema.". Calif Med. 89 (6): 390–3. PMC 1512545Freely accessible. PMID 13608293. 
  16. JANSEY F, SZANTO PB, WRIGHT A (1957). "Postmastectomy lymphangiosarcoma in elephantiasis chirurgica; Stewart and Treves syndrome.". Q Bull Northwest Univ Med Sch. 31 (4): 301–7. PMC 3803612Freely accessible. PMID 13494640. 
  17. 17.0 17.1 Mackenzie DH (1972). "Lymphangiosarcoma.". J Clin Pathol. 25 (3): 273. PMC 477281Freely accessible. PMID 16811066. 
  18. Cozen W, Bernstein L, Wang F, Press MF, Mack TM (1999). "The risk of angiosarcoma following primary breast cancer.". Br J Cancer. 81 (3): 532–6. PMC 2362921Freely accessible. PMID 10507781. doi:10.1038/sj.bjc.6690726. 
  19. Danese CA, Grishman E, Dreiling DA (1967). "Malignant vascular tumors of the lymphedematous extremity.". Ann Surg. 166 (2): 245–53. PMC 1477364Freely accessible. PMID 6029576. 
  20. Taşdemir A, Karaman H, Ünal D, Mutlu H (2015). "Stewart-Treves Syndrome after Bilateral Mastectomy and Radiotherapy for Breast Carcinoma: Case Report.". J Breast Health. 11 (2): 92–94. PMC 5351494Freely accessible. PMID 28331699. doi:10.5152/tjbh.2015.1604. 
  21. TAYLOR GW (1959). "[Not Available].". Postgrad Med J. 35 (399): 2–7. PMC 2501941Freely accessible. PMID 13623542. 
  22. Parthiban R, Kaler AK, Shariff S, Sangeeta M (2013). "Squamous cell carcinoma arising from congenital lymphedema.". SAGE Open Med Case Rep. 1: 2050313X13496507. PMC 4857268Freely accessible. PMID 27489627. doi:10.1177/2050313X13496507. 
  23. "Lymphangiosarcoma in Chronic Lymphedematous Extremities.". Can Med Assoc J. 87 (4): 192. 1962. PMC 1849463Freely accessible. PMID 20327191. 
  24. McSwain B, Stephenson S (1960). "Lymphangiosarcoma of the Edematous Extremity.". Ann Surg. 151 (5): 649–56. PMC 1613712Freely accessible. PMID 17859624. 
  25. Cui L, Zhang J, Zhang X, Chang H, Qu C, Zhang J; et al. (2015). "Angiosarcoma (Stewart-Treves syndrome) in postmastectomy patients: report of 10 cases and review of literature.". Int J Clin Exp Pathol. 8 (9): 11108–15. PMC 4637645Freely accessible. PMID 26617830. 
  26. 26.0 26.1 26.2 Stanczyk M, Gewartowska M (2014). "Stewart-Treves syndrome.". Indian J Med Res. 139 (1): 179. PMC 3994737Freely accessible. PMID 24604055. 

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