Angiosarcoma lymphedema associated C44.-

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Angiosarcoma in chronic lymphostasis; Lymphangiosarcoma; Lymphedema associated angiosarcoma; Postmastectomy lymphangiosarcoma; Stewart-Treves Syndrome

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Löwenstein, 1906; Stewart and Treves, 1948

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Originally classified as lymphangiosarcoma, this rare clinical variant of angiosarcoma manifests itself in areas of chronically persistent lymphedema of quite different genesis, and tends to metastasize early.

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Rarely. About 300 cases have been described in the literature, mostly as post-mastectomy syndrome. The relative risk of lymphangiosarcoma in mastectomized patients is about 0.001%.

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  • Whether (lymphatic) angiosarcomas originate from endothelial cells of the blood or lymph vessels is a controversial issue.
  • Originally the syndrome was defined as lymphangiosarcoma after mastectomy for breast cancer! According to more recent approaches, it is considered a rare late complication of chronic lymphedema, independent of its genesis.
  • Causes discussed include post-traumatic conditions, postoperative etiology (post-mastectomy syndrome), postoperative radiation, idiopathic, congenital or parasitic (e.g. in filariasis) etiology.

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5-30 years after mastectomy (average 10-11 years). Average age of manifestation according to professional literature: 65th-70th LJ.

Clinical features
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Rough, initially light red, later deep red erythema that develops into hemorrhagic papules, plaques or nodules with a tendency to ulceration. Absence of or only slight pain. Prolonged persistence leads to confluent red-livid or blue-livid, coarse-elastic plaques or to disseminated seeding of the nodules over the affected extremity. In a later stage, tendency to extensive ulceration.

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  • Vascular spaces with atypical endothelial cells; inflammatory infiltrates, hemosiderin storage. Irregularly anastomosing, diffusely infiltrating vessels.
  • Immunohistology: UEA 1, vimentin and HLA-DR positive.

Differential diagnosis
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Skin metastasis; AIDS-associated Kaposi sarcoma.

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Due to the rarity of the disease there is no therapeutic consensus.

General therapy
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Intensive treatment of lymphedema (a major predisposing factor for angiosarcoma) with intermittent mechanical lymphatic drainage, compression therapy, fitting of an elastic arm stocking.

Radiation therapy
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For inoperable tumours without distant metastases, soft X-ray therapy (ED: 4-5 Gy; GD: 50-60 Gy) or fast electrons (ED: 2-4 Gy; GD: 50-60 Gy).

Internal therapy
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Cytostatics: In case of operable tumour and presence of distant metastases (e.g. Adramycin, Ifospamide), followed by radical resection of the tumour. The effectiveness of adjuvant therapies is controversial. Therapy approaches with liposomal encapsulated doxorubicin (20 mg/m2 KO, shock therapy every 14 days) have been described as successful. Isolated limb perfusion is considered an optional procedure ( TNF-α, melphalan).

Operative therapie
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The first choice therapy is the surgical removal of the tumour. Cave! Primary tumor is often multicenter. The radicality of the procedure is determined by the tumor stage. If no distant metastases are present at the time of diagnosis, a radical procedure with removal of the entire affected muscle group offers the best chances of survival. If the soft parts of the forearm are affected, amputation of the entire extremity is recommended, while additional disarticulation is recommended in the case of upper arm involvement.

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After 1.5 years, only about 50% of the patients survive regardless of the therapy. The 5 year survival rate in relevant studies was 13.6%. Untreated patients usually die after 5-8 months after dissemination and distant metastases (lung metastases). With treatment, the survival time increases to 20 months.

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  1. Cui L et al (2015) Angiosarcoma (Stewart-Treves syndrome) in postmastectomy patients: report of 10 cases and review of literature. Int J Clin Exp Pathol 8:11108-1115.
  2. Goetze S et al (2004) Successful therapy of Stewart-Treves syndrome with liposomal encapsulated doxorubicin. JDDG 2: 49-52
  3. Harrison WD et al (2015) Stewart-Treves syndrome following idiopathic leg lymphoedema: remember sarcoma. J Wound Care 24(6 Suppl):S5-7.
  4. Komorowski AL et al (2003) Angiosarcoma in a chronically lymphedematous leg: an unusual presentation of Stewart-Treves syndrome. South Med J 96: 807-808
  5. Löwenstein S (1906) The etiological connection between acute single trauma and sarcoma. Contribution by the surgeon 48: 780
  6. Mentzel T et al (2002) Tumors of the lymphatic vessel of the skin and soft tissue. Pathologist 23: 118-127
  7. Nakamura Y et al (2015) Long term control of pleural metastasis in Stewart-Treves syndrome with single-agent chemotherapies followed by maintenance chemotherapy. JDDG 13: 818-820
  8. Pitche P et al (2002) Stewart-Treves' syndrome: long term survival] Ann Dermatol Venereol 129: 236-237
  9. Stewart FW, Treves N (1948) Lymphangiosarcoma in postmastectomy lymphedema: a report of 6 cases in elephantiasis chirurgica. Cancer 1: 64-81
  10. Tse TS, Cooper LT (2001) Images in vascular medicine. Stewart's Treves angiosarcoma. Vasc Med 6: 267-268


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Last updated on: 29.10.2020