The term "lymphangiosarcoma" is controversial. While immunohistochemical stains for lymphatic-based antibodies are positive in the majority of cases of Stewart-Treves syndrome and in angiosarcomas induced by radiotherapy, electron microscopy reveals the cells to be surrounded by structures more consistent with a vascular origin (complete basal lamina, intercellular junctions, etc). Use of the term may help distinguish LAS from the more common form of cutaneous angiosarcoma (cAS) that affects the head and neck region of elderly patients. While the exact etiology of LAS is unknown, it is thought that postoperative hematoma formation, chronic infections, tissue fibrosis, and even local radiation may all create a regional immunodeficient state contributing to the development of LAS.
Acute (less than 18 months) and chronic (more than 18 months) lymphedema in the upper extremities occurs in up to 35% of patients following radical mastectomy with axillary lymph node dissection or radiotherapy. LAS has been reported to occur in less than 1% of women at 5 and 15 years postradical 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 postsurgical lymphedema and cases of LAS.
LAS metastases most frequently go to the thoracic cavity and lungs but may also involve other internal organs and bone. Prognosis for LAS is extremely poor, as it is associated with rapid progression and metastases. Therapeutic options include chemotherapy and/or radiation therapy and limb amputation; however, none is considered effective. Five-year survival rates have been reported to be less than 10%.
Pediatric patient considerations: LAS can rarely occur with congenital lymphedema (ie, Milroy disease).
C49.10 – Malignant neoplasm of connective and soft tissue of unspecified upper limb, including shoulder
403986008 – Lymphangiosarcoma
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-cell lymphomas 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 herpesvirus type 8 antibodies. Not consistently associated with lymphedema.
- 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 the 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.