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. 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. Lymphangiosarcoma is a rare entity and 300 cases of lymphangiosarcoma after breast cancer have been reported 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 compared to males.[31]

Race

  • There is no racial predilection for lymphangiosarcoma.

Risk Factors

Common risk factors in the development of lymphangiosarcoma include:[8][32][28][33][34][35][36]

Natural History, Complications and Prognosis

Natural History

Complications

Prognosis

  • Prognosis is generally poor, and the 5 year survival rate of patients with lymphangiosarcoma is less than 5%.[41][42]

Diagnosis

Symptoms

Symptoms of lymphangiosarcoma may include the following:[43]

Physical Examination

Physical examination may be remarkable for:[44][45][46]

Laboratory Findings

Imaging Findings

  • MRI with intravenous contrast is the imaging modality of choice to asses the local extent 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

Treatment

Medical Therapy

Surgery

  • Amputation of the affected limb is the most common approach to the treatment of lymphangiosarcoma.[47]

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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  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.
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