Lymphangioma, occuring as a rare long-term complication of chronic lymphedema, has been described in association with scarring process including recurrent infections, radiotherapy, scrofuloderma, scleroderma, keloid, and tumors. Only one case has been reported in the patient with congenital lymphedema in English literature. The case discussed in this report developed 40 years after following congenital lymphedema. Both this condition and lymphangiosarcoma, its malignant counterpart, may arise in lymphedematous extremities regardless of the etiology of lymphatic obstruction.
Cicatrix ; Extremities ; Humans ; Keloid ; Lymphangioma ; Lymphangiosarcoma ; Lymphedema* ; Radiotherapy ; Tuberculosis, Cutaneous

Since the entity of postmastectomy lymphangiosarcoma was first reported by Stewart and Traves in 1948, postmastectomy lymphangiosarcoma has become a well recognized, uncommon malignant tumor which occurs in the upper extremity following mastectomy for mammary carcinoma. The postmastectomy lymphangiosarcoma occurred at an average age of 63.9 years and at an average of 10 years and 3 months following mastectomy. The lymphangiosarcoma raised from blood and lymphatic vessel. The histologic appearance has been observed edematous dermis and dilated lymphatics lining with malignant cells. Most authors recommend radical amputation for treatment, either shoulder disarticulation or forequarter amputation. Other modalities of treatment including radiotherapy were considered as ineffective. The present report provides a case of the regression of postmastectomy lymphangiosarcoma with chronic lymphedima by external irradiation. Radiation therapy was used as primary therapy. Total tumor dose of 6500 cgy in 9 weeks was delivered using 6 MV x-ray and 8 MeV electron.
Amputation ; Dermis ; Disarticulation ; Lymphangiosarcoma* ; Lymphatic Vessels ; Lymphedema ; Mastectomy ; Radiotherapy ; Shoulder ; Upper Extremity

Primary angiosarcoma of the breast is extremely rare and is observed in 0.0005% to 0.05% of primary breast tumor cases. The diagnosis of this tumor is difficult due to its undefined characteristics. Radiologic findings are often nonspecific and appear completely normal in one-third of patients with primary angiosarcomas. The prognosis is usually poor, and the treatment choices include mastectomy or wide excision. Radiotherapy and chemotherapy produce varying results. We report a patient with primary angiosarcoma of the breast to further our understanding of the characteristics of this tumor and facilitate the correct diagnosis of breast angiosarcoma.
Breast Neoplasms ; Breast* ; Diagnosis ; Drug Therapy ; Hemangiosarcoma* ; Humans ; Lymphangiosarcoma ; Mastectomy ; Prognosis ; Radiotherapy ; Sarcoma

BACKGROUNDThe development of angiosarcoma in oedematous tissue is referred to as Stewart-Treves syndrome (STS). This rare and fatal complication is associated with chronic post mastectomy lymphoedema and radiotherapy for breast cancer. Angiosarcoma spread is facilitated by the formation of blood vessels (angiogenesis) and lymph vessels (lymphangiogenesis). In the future antiangiogenic therapy may improve the poor outcome of current treatments. There was evidence that blocking the angiogenenesis would inhibit progression of angiosarcoma. It seems reasonable to hypothesize that blocking the lymphangiogenesis may yield similar results. Although angiosarcomas commonly derive from blood vessels, in case of STS angiosarcomas chronic lymphoedema may suggest its lymphatic origin. The goal of this study was to visualize interstitial space and lymphatics in the central and peripheral regions of STS angiosarcoma.

METHODSOn tissue samples obtained from STS angiosarcoma we have performed: first colour stereoscopic lymphography to visualise the morphology of lymphatic vessels and extracellular spaces, second immunohistochemical staining specific for lymphatic vessels endothelium (LYVE-1) and blood endothelial cells (CD31, factor VIII) and prolymphangiogenic vascular endothelial growth factor (VEGF-C) for precise identification of lymphatic endothelia. STS angiosarcoma morphology was assessed by comparison of pictures obtained on lymphography, microscopy and confocal microscopy.

RESULTSSTS angiosarcomas present heterogenous morphology with areas dominated by hemangiosarcoma and lymphangiosarcoma structures. STS angiosarcoma expressed phenotypes of both blood and lymphatic endothelia. LYVE-1 and VEGF-C is expressed by STS angiosarcoma and may be used to discriminate tumour differentiation. Morphology of lymphatic vessels and spaces in the tumour suggest absence of their normal lymphatic function.

CONCLUSIONSOur results confirmed both hemangio- and lymphangiogenic origin of STS angiosarcoma. Expression of VEGF-C makes STS angiosarcoma a good candidate for targeted antilymphangiogenic therapy. However, morphology of intratumoral lymphatics on colour lymphography suggested their impaired function, which can hamper drug distribution.

Aged ; Female ; Hemangiosarcoma ; blood supply ; drug therapy ; pathology ; Humans ; Immunohistochemistry ; Lymphangiogenesis ; Lymphangiosarcoma ; blood supply ; drug therapy ; pathology ; Lymphatic Vessels ; pathology ; Lymphography ; Microscopy, Confocal ; Phenotype