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Lymphangiosarcoma - Skin
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Lymphangiosarcoma - Skin

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Contributors: Patricia Ting MD, Ben Barankin MD, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Lymphangiosarcoma (LAS), also known as Stewart-Treves syndrome (lymphangiosarcoma, angiosarcoma, or hemangiosarcoma post-mastectomy), is a rare, aggressive cutaneous angiosarcoma occurring in association with chronic lymphedema. It was originally described in 1948 by Stewart and Treves in six patients with upper extremity lymphedema post-mastectomy. LAS usually affects the upper extremities following axillary lymph node dissection or local radiation therapy for breast carcinoma. Metastases most frequently go to the thoracic cavity and lungs but may also involve other internal organs and bone. LAS has a poor prognosis.

Acute (<18 months) and chronic (>18 months) lymphedema in the upper extremities occurs in 10-35% of patients following radical mastectomy with axillary lymph node dissection or radiotherapy. LAS has been reported to occur in <1% of women between 5 and 15 years post-radical mastectomy with lymphedema. Advances in surgical management of breast carcinoma, including partial mastectomies, sentinel lymph node biopsies, and adjuvant radiation therapy, have significantly decreased the incidence of associated post-surgical lymphedema and cases of LAS. Isolated cases of LAS have also been associated with secondary forms of lymphedema (eg, idiopathic, congenital, trauma induced, morbid obesity, venous stasis, filarial, etc) in humans and animals.

The term lymphangiosarcoma is a misnomer, as the malignant cells of origin arise from vascular endothelial cells rather than from the lymphatics and, therefore, angiosarcoma or hemangiosarcoma would be more appropriate descriptive terms. While the exact etiology of LAS is unknown, it is thought that post-operative hematoma formation, chronic infections, tissue fibrosis, and even local radiation may all create a regional immunodeficient state contributing to the development of LAS.

Pediatric Patient Considerations
LAS can occur with congenital (eg, Milroy's) lymphedema.

Codes

ICD10CM:
C49.10 – Malignant neoplasm of connective and soft tissue of unspecified upper limb, including shoulder

SNOMEDCT:
403986008 – Lymphangiosarcoma

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Cutaneous leukemia – rare; immunohistochemical stain positive for human T-cell leukemia virus type 1 (HTLV-1).
  • Cutaneous lymphoma (B- or T-cell) – differentiate B- and T-cell lymphomas by immunohistochemical markers, molecular gene rearrangement studies for T-cell lymphomas.
  • Kaposi sarcoma – look for rapidly growing violaceous to dark brown papules, plaques, or nodules on the lower extremities, face, oral mucosa and genitalia; immunohistochemical stain positive for human herpes virus 8 antibody.
  • Lymphocytoma cutis – reactive process to various factors including infection, vaccination, medications, trauma, arthropod bites, tattoos, etc; look for mixed inflammatory infiltrate on skin biopsy.
  • Lymphomatoid papulosis – look for crops of red-brown papules, pustules, or papulonodules on trunk and extremities that may resolve spontaneously; immunohistochemical stain CD30 positive.
  • Metastatic carcinoma – look for another primary malignancy associated with cutaneous metastases (eg, melanoma, breast, lung, renal).
  • Merkel cell carcinoma – look for rapidly growing asymptomatic red, blue, or skin-colored papules on sun-exposed areas of the head, neck, and legs; immunohistochemical stain cytokeratin (CK) 20 positive.

Best Tests

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Management Pearls

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Therapy

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References

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Last Updated: 01/23/2015
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Lymphangiosarcoma - Skin
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Lymphangiosarcoma : Purple color, Upper extremity edema, Smooth papules, Therapeutic radiation exposure, Tender skin lesions
Clinical image of Lymphangiosarcoma
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