Cases reported "Lymphedema"

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1/39. Stewart-Treves syndrome: lymphangiosarcoma following mastectomy.

    lymphangiosarcoma (LAS) is an aggressive, malignant vascular tumor following long-lasting chronic lymphedema. patients with LAS demonstrate a history of breast cancer treated by radical mastectomy in the majority of patients. In the 1960s the incidence of LAS in patients with a 5-year survival after radical mastectomy varied from 0.07 to 0.45%. Today, due to changes in the operative techniques of breast cancer, less chronic lymphedema is seen with only a scant number of LAS patients. The etiology of this enigmatic tumor is not yet completely understood. Histologically, LAS arises from vascular endotheliocytes, and all vascular sarcomas originating in the setting of a chronic lymphedema are categorized as LAS. There is no standard treatment of LAS. The treatment options include radical ablative surgery, radiation therapy, and chemotherapy. The prognosis of LAS is poor; long-term survival is the exception. Only early recognition and radical surgery offer a chance of cure.
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2/39. hemangiosarcoma of the left hand in a patient with the rare combination of Maffucci's and Stewart Treves syndrome.

    We describe a patient with the previously unseen combination of Maffucci's and Stewart Treves syndrome who presented with an angiosarcoma of the hand. Maffucci's syndrome is characterized by the presence of multiple enchondroma and soft tissue hemangioma. The syndrome is a rare nonhereditary condition with a usual onset in childhood. Malignant transformations are a common feature of this syndrome. In 1948, Stewart and Treves first described six cases of lymphangiosarcoma after radical mastectomy. This syndrome is an unusual form of angiosarcoma occuring as a complication of lymphedema. Chronic lymphedema and lymphangiectasia preceding lymphangiosarcoma may not only be induced by radical mastectomy with axillary lymph node dissection and postoperative radiation therapy. Posttraumatic, congenital or spontaneous chronic lymphedema may also be associated with lymphangiosarcoma. A time interval of many years seems to be required before malignant transformation develops. Generally the syndrome has a very poor prognosis. Both syndromes described above are of a rare frequency. We report this case because of prior unknown coincidence of both syndromes.
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3/39. Lipedema complicated by lymphedema of the abdominal wall and lower limbs.

    We describe a 52 year-old woman in whom lymphedema primarily of the abdominal wall was superimposed on lipedema resulting in an abdomen of enormous dimensions with marked impairment of ambulation. Treatment consisted of preoperative compression of the legs by an external pneumatic device (Lympha-Press) followed by excision of the lymphedematous abdominal fat pad in conjunction with "debulking" of the right leg. The patient illustrates the extremes of lipedema complicated by lymphedema and the technical difficulties associated with its management.
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4/39. Successful mapping of lymphorrhea using patent blue dye after lymph node dissection for malignant melanoma.

    Patent blue is a dye that has been used for intraoperative lymphatic mapping. We used this mapping method on a patient with lymphorrhea after groin dissection. We easily detected the lymphatic channel causing lymphorrhea and successfully ligated it. This technique may have great merit for treating of lymphorrhea.
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5/39. Cutaneous angiosarcoma in an irradiated breast after breast conservation therapy for cancer: association with chronic breast lymphedema.

    The authors report a rare case of cutaneous angiosarcoma arising more than 5 years after excision of a 1.5 cm invasive ductal cancer of the breast. All lymph nodes were negative for metastatic breast cancer in this 68-year-old female. The patient had postoperative therapy consisting of 5040 cGy over a 5 week period using a 6 Megavolt linear accelerator. After radiation therapy to the breast and axillae, the patient developed chronic hard, taut edema of the irradiated right breast. tamoxifen was administered for 5 years and then stopped. Three months after the cessation of tamoxifen, cutaneous angiosarcoma was found by skin biopsy. A complete mastectomy removed all tumor with clear margins. There are less than 60 cases of radiation associated breast angiosarcoma found in the literature. The presence of chronic lymphedema in the breast after radiation therapy possibly contributes to the development and is an early warning sign for later development of secondary angiosarcoma. The characteristic purple nodules and discoloration of the irradiated skin is the hallmark to suspect the diagnosis. The authors recommend long-term clinical surveillance for this tumor for all patients who have received breast conservative surgical therapy with concomitant radiation therapy for primary breast cancer.
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6/39. Supermicrosurgical lymphaticovenular anastomosis for the treatment of lymphedema in the upper extremities.

    Over the last eight years, the authors analyzed obstructive lymphedema of a unilateral upper extremity in a total of 27 females, comparing the use of supramicrosurgical lymphaticovenule anastomoses and/or conservative treatment. The most common cause of edema was mastectomy, with or without subsequent radiation therapy for breast cancer. As an objective assessment of the extent of edema, the circumferences of the affected and opposite normal forearms were measured at 10 cm below the olecranon of the arm. Twelve of these patients received continual bandaging. In these patients, the average excess circumference of the affected arm was 6.4 cm over that of the normal forearm; the average duration of edema before treatment was 3.5 years; the average period for conservative treatment was 10.6 months; and the average decrease in circumference was 0.8 cm (11.7 percent of the preoperative excess). Twelve patients underwent surgery and postoperative continual bandaging. In these patients, the average excess circumference was 8.9 cm; the average duration of edema before surgery was 8.2 years; the average follow-up after surgery was 2.2 years; and the average decrease in circumference was 4.1 cm (47.3 percent of the preoperative excess). These results indicated that supermicrolymphaticovenular anastomoses with postoperative bandaging have a valuable place in the treatment of obstructive lymphedema.
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7/39. Congenital balloon digits in two neonates caused by constriction rings.

    Balloon digits were found in two neonates with congenital constriction ring syndrome. The affected digits were the right long finger and right great toe. They were surgically treated at the age of 10 and 9 days, respectively. Morphologic improvement was dramatic after surgery. In cases with extensive enlargement, severe cyanosis, redness, and no subsidence of edema within several days after birth, early operative treatment may be necessary to maintain digit viability and prevent autoamputation due to circulatory embarrassment. It can also be helpful to prevent fibrosis of the subcutaneous tissue. Pathologic examination revealed marked proliferation of fibrous tissue and lymphatic vessels.
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8/39. Post-therapeutic lymphedema: scintigraphy before and after autologous lymph vessel transplantation: 8 years of long-term follow-up.

    The function of lymphatic vessel grafts was proved in 12 patients with lymphedema of the upper extremity by visual and semiquantitative evaluation of a preoperative baseline study and scintigraphic follow-up data for a period of 8 years after microsurgical treatment. The transplantation site was an upper extremity. In 11 of 12 patients, lymphatic function improved after autologous lymphatic vessel transplantation compared with preoperative findings. This could be verified by a statistically significant decrease of the transport index ( < 0.01), clear demonstration of lymph nodes, and a less diffuse distribution pattern of the Tc-99m-labeled nanocolloids. In three patients, the vessel graft could be detected by scintigraphy. lymphoscintigraphy combined with semiquantitative evaluation of lymphatic transport kinetics has been shown to be an easy and reliable method to assess lymphatic function before and after autologous lymph vessel transplantation.
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9/39. Follow-up study of upper limb lymphedema patients treated by microsurgical lymphaticovenous implantation (MLVI) combined with compression therapy.

    We present a follow-up study of 18 patients with upper limb lymphedema treated by microsurgical lymphaticovenous implantation (MLVI) combined with compression therapy. This combined technique provides increased lymphatic flow through newly created lymphaticovenous bypasses by the MLVI surgery, with assistance for pumping function in the lymphatics by compression therapy. Preoperative assessment of the affected limb was performed by the average enlargement of edema circumference (AEEC), comparing the lymphedema limb and normal limb circumferences. Objective improvement was analyzed by the percent reduction of edema circumference (%REC) at two levels of the lymphedema limb. With an average follow-up of 24 months, 77.8% of patients presented excellent or good results, with %REC >50% at either the distal or proximal site of the treated limb. This combined treatment can be expected to provide favorable long-term results, even for patients with AEEC >8 cm.
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10/39. sentinel lymph node biopsy lowers the rate of lymphedema when compared with standard axillary lymph node dissection.

    Arm edema occurs in 20 to 30 per cent of patients who undergo axillary lymph node dissection (ALND) for carcinoma of the breast. sentinel lymph node biopsy (SLNB) in lieu of ALND for staging of breast cancer significantly lowers this morbidity. We hypothesized that SLNB would have a lower lymphedema rate than conventional axillary dissection. patients who underwent SLNB were compared with those who underwent level I and II axillary node dissection. A total of 125 patients were evaluated with 77 patients who underwent SLNB and 48 patients who underwent ALND. The arm circumference 10 cm above and 10 cm below the olecranon process was measured on both arms. In this series a difference in arm circumference greater than 3 cm between the operated and nonoperated side was defined as significant for lymphedema. lymphedema was seen in two of 77 (2.6%) patients in the SLNB group as compared with 13 of 48 (27%) ALND patients. Given the above data patients who underwent sentinel lymph node biopsy show a significantly lower rate of lymphedema than those who had axillary lymph node dissection. This has an important impact on long-term postoperative management of patients with breast cancer.
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