Lymphangiosarcoma

Abstract: This review presents the diagnostic features, the pathophysiology and the available therapies for lymphedema. This disease is often able to be diagnosed by its characteristic clinic- cal presentation, yet, in some cases, ancillary tests might be necessary to establish the diag- nosis, particularly in the early stages of the disease and in edemas of mixed etiology. These diagnostic modalities are also useful in clinical studies. Available modalities include isotopic lymphoscintigraphy, indirect and direct lymphography, magnetic resonance imaging, computed tomography and ultrasonography. Lymphedema may be primary or secondary to the presence of other diseases and/or to the consequences of sur- gery. Primary lymphedema may occur at any phase of life but it most commonly appears at puberty. Secondary lymphedema is encountered more often. The most prevalent worldwide cause of lymphedema is filariasis, which is particularly common in south-east Asia. In the USA, postsurgical lymphedema of the extremity prevails. Complications of chronic limb lymphedema include recurrent cellulitis and lymphangiosarcoma. Most patients are treated conservatively, by means of various forms of compression therapy, including complex physical therapy, pneumatic pumps and compressive garments. Volume reducing surgery is performed rarely. Lymphatic microsurgery is still in an experimental stage, although a few centers consistently report favorable outcomes.

Abstract: Between 1954 and 1983, 7620 patients were treated for breast carcinoma at Institut Gustave Roussy (France). Of these patients, 6919 were followed for at least 1 year. Out of these, 11 presented with sarcomas thought to be induced by irradiation, 2 of which were Steward-Treves Syndrome, and 9 of which were sarcomas within the irradiated fields. All histological slides were reviewed and a comparison with those of breast cancer was done. The sites of these sarcomas were: parietal wall, 1 case; second costal cartilage, 1 case; infraclavicular region, 1 case; supraclavicular region, 2 cases; internal third of the clavicle, 2 cases; axillary region 2 cases; and the internal side of the upper arm (Stewart-Treves syndrome), 2 cases. The median age of these 11 patients at the diagnosis of sarcomas was 65.8 (49-83). The mean latent period was 9.5 years (4-24). Three patients underwent radical mastectomy and nine modified radical mastectomy. Only one patient received chemotherapy. The radiation doses received at the site of the sarcoma were 45 Gy/18 fr. for 10 cases and 90-100 Gy for 1 case (due to overlapping between two fields). The histology was as follows: malignant fibrous histiocytoma, 5 cases; fibrosarcoma, 3 cases; lymphangiosarcoma, 2 cases; and osteochondrosarcoma, 1 case. The median survival following diagnosis of sarcoma was 2.4 years (4 months-9 years). Two patients are still alive: one with recurrence of her breast cancer, the other in complete remission, with 7 and 3 years follow-up, respectively. All other patients died from their sarcomas. The cumulative incidence of sarcoma following irradiation of breast cancer was 0.2% (0.09-0.47) at 10 years. The standardized incidence ratio (SIR) of sarcoma (observed n# of cases (Obs)/expected n# of cases (Exp) computed from the Danish Cancer Registry for the same period) was 1.81 (CI 0.91-3.23). This is significantly higher than one, with a p = 0.03 (One Tailed Exact Test). The mean annual excess (Obs-Exp)/100.000 person-years at risk during the same period/(100,000) was 9.92. This study suggests that patients treated by radiation for breast cancer have a risk of subsequent sarcomas that is higher than the general population. However, the benefit from adjuvant radiation therapy in the treatment of breast cancer exceeds the risk of second cancer; therefore, the potential of radiation-induced sarcomas should not be a factor in the selection of treatment for patients with breast cancer.

Abstract: Data from 23 cases of lymphangiosarcoma associated with chronic lymphedema seen at the Mayo Clinic and 163 cases from the world literature are reviewed. The available information in 162 cases of postmastectomy lymphedematous lymphangiosarcoma revealed that the lesion occurred at an average age of 63.9 years (range 44 to 84 years) and an average of 10 years 3 months after mastectomy. Fifty per cent of the 129 patients eligible for follow-up were dead within 19 months after treatment, and only 11 patients have survived 5 years or more. Amputation gave a slightly better prognosis than did radiation therapy. Of 24 patients (two from the Mayo Clinic) with lymphangiosarcoma and non-postmastectomy lymphedema, 12 (50%) were dead within 34 months after treatment. Only two of the patients were long-term survivors (5 and 15 years), and both were treated by amputation. Early recognition of the lesion and prompt radical ablative surgery seem to offer the best chance for survival.

Abstract: Information is presented on eleven Mayo Clinic patients with postmastectomy lymphangiosarcoma and on two patients with lymphangiosarcoma in lymphedematous extremities in whom malignant disease was unrelated to surgical treatment One of the latter two tumors occurred in the arm of a patiemit with congenital lymphedema, and the other in the leg of a patient with long-standing idiopathic lymphedema Also, the clinical and pathological findings in forty-seven previously reported cases of lymphangiosarcoma occurring in postmastectomy lymphedema are summarized In addition, data on four previously reported examples of lymphangiosarcoma occurring in chronic lymphedema of the leg are given briefly Finally, reports on seven cases of probable lymphangiosarcoma are reviewed The data on the sixty-four accepted examples and on the seven probable examples of this condition are compared The severe lymphangiomatosis and endothelial hyperplasia of the lymphatic spaces of the subcutaneous tissues would appear to herald the onset and to account for the usual widespread occurrence of the lesions This latter characteristic, exhibited in the majority of the patients, would appear to explain the poor results of treatment Despite varying forms and combinations of radical treatment, only twelve of fifty-six patients with postmastectomy lymphedema were alive when last traced Six of these twelve were known to have had one or more local recurrent or metastatic lesions Only three of these patients are recorded as surviving after the third year following onset of the disease, one for a period of approximately four years, one for eleven years, and one for twelve years Of the forty-four patients known or presumed to be dead (six presumed dead), only six survived for more than three years after onset of the disease One died forty-two months later of a cerebral vascular accident, and one fifty-two months later by suicide There was no clinical evidence of lymphangiosarcoma in either patient at the time of death