: Hemangioendotheliomas are vascular neoplasms occupying a spectrum of biological potential ranging from benign to low-grade malignancy. Composite hemangioendothelioma (CH) is one of the less commonly encountered variants exhibiting a mixture of elements of other hemangioendothelioma subtypes, such as epithelioid, retiform, and spindle cell. Some authors have identified areas histopathologically equivalent to angiosarcoma within CH, raising the question of the true nature of this neoplasm. Although CH recurs locally, there are only 3 reported cases which metastasized. To date, 26 cases (including the present case) have been described in the literature. Herein, we describe a unique case of CH arising in the background of previous radiation therapy and long-standing lymphedema (classically associated with the development of angiosarcoma-Stewart-Treves syndrome) that harbored higher grade areas but behaved as a low-grade malignant neoplasm. This, in conjunction with the many reported cases of CH-harboring angiosarcoma-like areas, and the occasional association with a history of lymphedema, raises the question of whether this variant of hemangioendothelioma may actually be an angiosarcoma that behaves prognostically better than the conventional type. After careful study of the natural disease progression of the current case and review of the literature, we discuss justification for the continued classification of CH as a low-grade malignancy.

Lymphatic filariasis (LF) is a vector-borne parasitic disease that can clinically manifest as disabling lymphedema. Although the LF elimination program aims to reduce disability and to interrupt transmission, there has been a scarcity of disease morbidity management programs, particularly on a national scale. This report describes the implementation of the first nationwide LF lymphedema management program. The program, which was initiated in Togo in 2007, focuses on patient behavioral change. Its goal is two-fold: to achieve a sustainable program on a national-scale, and to serve as a model for other countries. The program has five major components: 1) train at least one health staff in lymphedema care in each health facility in Togo; 2) inform people with a swollen leg that care is available at their dispensary; 3) train patients on self-care; 4) provide a support system to motivate patients to continue self-care by training community health workers or family members and providing in home follow-up; and 5) integrate lymphedema management into the curriculum for medical staff. The program achieved the inclusion of lymphedema management in the routine healthcare package. The evaluation after three years estimated that 79% of persons with a swollen leg in Togo were enrolled in the program. The adherence rate to the proposed World Health Organization treatment of washing, exercise, and leg elevation was more than 70% after three years of the program, resulting in a stabilization of the lymphedema stage and a slight decrease in reported acute attacks among program participants. Health staff and patients consider the program successful in reaching and educating the patients. After the external funding ended, the morbidity management program is maintained through routine Ministry of Health activities.

BACKGROUND:

Axillary lymph node dissection (ALND) is recommended for patients with clinically node-positive breast cancer and carries a risk of lymphedema >30 %. Patients with node-positive breast cancer may consider neoadjuvant chemotherapy, which can reduce node positivity. We sought to determine if neoadjuvant chemotherapy reduced the risk of lymphedema in patients undergoing ALND for node-positive breast cancer.

METHODS:

The 229 patients who underwent unilateral ALND and chemotherapy were divided into two groups: 30 % (68/229) had neoadjuvant and 70 % (161/229) had adjuvant chemotherapy. Prospective arm volumes were measured via perometry preoperatively and at 3- to 7-month intervals after surgery. Lymphedema was defined as relative volume change (RVC) ≥10 %, >3 months from surgery. Kaplan-Meier curves and multivariate regression models were used to identify risk factors for lymphedema.

RESULTS:

Fifteen percent (10/68) of neoadjuvant patients compared with 23 % (37/161) of adjuvant patients developed RVC ≥10 % (hazard ratio = 0.76, p = 0.39). For all patients, body mass index was significantly associated with lymphedema (p = 0.0003). For neoadjuvant patients, residual lymph node disease after chemotherapy was associated with a ninefold greater risk of lymphedema compared to those without residual disease (p = 0.038).

CONCLUSIONS:

Patients who underwent neoadjuvant chemotherapy did not have a statistically significant reduction in risk of lymphedema. Among patients who receive neoadjuvant chemotherapy, residual lymph node disease predicted a greater risk of lymphedema. These patients should be closely monitored for lymphedema and possible early intervention for the condition.

BACKGROUND:

Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease predominantly affecting young women. Some of these patients develop lymphedema of the lower extremities and buttocks; however, neither the exact frequency of LAM-associated lymphedema nor the clinical features of such patients is well delineated.

OBJECTIVES:

To document the frequency, features, and treatment of LAM-associated lymphedema.

METHODS:

We reviewed all medical records of patients listed in the Juntendo University LAM registry for the 30 years preceding August 2010.

RESULTS:

Of 228 patients registered with a diagnosis of LAM, eight (3.5%) had LAM-associated lymphedema of the lower extremities. All were females with sporadic LAM, and their mean age when diagnosed was 32.5 years (range 23-44). Lymphedema of the lower extremities was the chief or a prominent presenting feature in five of these LAM patients. CT scans showed that all eight patients had enlarged lymph nodes (lymphangioleiomyomas) in the retroperitoneum and/or pelvic cavity. Yet, cystic destruction of the lungs was mild in four patients, moderate in two and severe only in two. Seven of these patients were treated by administering a fat-restricted diet and complex decongestive physiotherapy, and four received a gonadotropin-releasing hormone analog. With this combined protocol, all eight patients benefitted from complete relief or good control of the lymphedema.

CONCLUSIONS:

Lymphedema is a rare complication of LAM and may be associated with axial lymphatic involvement or dysfunction rather than severe cystic lung destruction. The combined multimodal treatments used here effectively resolved or controlled LAM-associated lymphedema.

OBJECTIVE:

To examine factors associated with variations in diagnosis and rehabilitation treatments received by women with self-reported lymphedema secondary to breast cancer care.

DESIGN:

Population-based, prospective study.

SETTING:

California, Florida, Illinois, New York.

PARTICIPANTS:

Elderly (65+) women identified from Medicare claims as having had an incident breast cancer surgery in 2003.

INTERVENTIONS:

N.A.

MAIN OUTCOME MEASURES:

Self-reported incidence of lymphedema symptoms, formal lymphedema diagnosis; treatments for lymphedema.

RESULTS:

Of the 450 breast cancer survivors with lymphedema who participated in the study, 290 (64.4%) were formally diagnosed with the condition by a physician. An additional 160 (35.6%) reported symptoms consistent with lymphedema (arm swelling on the side of surgery that is absent on the contralateral arm) but were not formally diagnosed. Of those reporting as being diagnosed by a physician, 39 (13.4%) received the comprehensive decongestive therapy (CDT) that included multiple components of treatment (manual lymphatic drainage (MLD), bandaging with short stretch bandages, using compression sleeves, skin care and remedial exercises), 24 (8.3%) received MLD only, 162 (55.9%) used bandages, compression garments or a pneumatic pump only, 8 (2.8%) relied solely on skin care or exercise to relieve symptoms and 65 (22.4%) received no treatment at all. Multivariate regressions revealed that race (African American), lower income, and lower levels of social support increased a woman's probability of having undiagnosed lymphedema. Even when formally diagnosed, African American women were more likely to receive no treatment or to be treated with bandages/compression only, rather than to receive the multi-modality, comprehensive decongestive therapy.

CONCLUSIONS:

Lymphedema is a disabling chronic condition related to breast cancer treatment. Our results suggest that a substantial proportion of those reporting symptoms were not formally diagnosed with the condition, thereby reducing their opportunity for treatment. The variation in rehabilitation treatments received by women who were formally diagnosis with the condition by a physician suggests that lymphedema might not have been optimally addressed in many cases despite availability of effective interventions.

The lymphatic vasculature is essential for fluid homeostasis and transport of immune cells, inflammatory molecules, and dietary lipids. It is composed of a hierarchical network of blind-ended lymphatic capillaries and collecting lymphatic vessels, both lined by lymphatic endothelial cells (LECs). The low hydrostatic pressure in lymphatic capillaries, their loose intercellular junctions, and attachment to the surrounding extracellular matrix (ECM) permit passage of extravasated blood plasma from the interstitium into the lumen of the lymphatic capillaries. It is generally thought that interstitial fluid accumulation leads to a swelling of the ECM, to which the LECs of lymphatic capillaries adhere, for example via anchoring filaments. As a result, LECs are pulled away from the vascular lumen, the junctions open, and fluid enters the lymphatic vasculature. The collecting lymphatic vessels then gather the plasma fluid from the capillaries and carry it through the lymph nodes to the blood circulation. The collecting vessels contain intraluminal bicuspid valves that prevent fluid backflow, and are embraced by smooth muscle cells that contribute to fluid transport. Although the lymphatic vessels are regular subject to mechanical strain, our knowledge of its influence on lymphatic development and pathologies is scarce. Here, we discuss the mechanical forces and molecular mechanisms regulating lymphatic vascular growth and maturation in the developing mouse embryo. We also consider how the lymphatic vasculature might be affected by similar mechanomechanisms in two pathological processes, namely cancer cell dissemination and secondary lymphedema.

Chronic ulceration can be complicated by development of a malignancy. The most frequent associated malignances are squamous cell carcinoma and basal cell carcinoma, although melanoma, leiomyosarcoma and adenocarcinoma are less commonly seen. Chronic lymphoedema may also predispose to development of some malignancies, including lymphangiosarcoma, squamous cell carcinoma and Kaposi's sarcoma. Here, we report the case of a 77-year-old man with primary lymphoedema, who developed melanoma in a chronic foot ulcer of 60 years' duration. The patient underwent wide excision for the melanoma, and remains free from metastases at 1-year follow up.

PURPOSE:

Physical activity (PA) is known to provide physical and mental health benefits to uterine cancer survivors. However, it is unknown if PA associates with lower limb lymphedema (LLL), an accumulation of protein-rich fluid in the lower limbs. Therefore, we sought to examine the association between PA and LLL in uterine cancer survivors, with a focus on walking.

METHODS:

We conducted a cross-sectional study using mailed surveys among uterine cancer survivors who received care at a university-based cancer center. We asked about PA, walking, and LLL symptoms using validated self-report questionnaires. PA was calculated using metabolic equivalent hours per week (MET-hrs[BULLET OPERATOR]wk), and walking was calculated using blocks per day (blocks[BULLET OPERATOR]d).

RESULTS:

The response rate to our survey was 43%. Among the 213 uterine cancer survivors in our survey, 36% were classified as having LLL. Compared with participants who reported <3 MET-hrs[BULLET OPERATOR]wk of PA, participants who reported ≥18.0 MET-hrs[BULLET OPERATOR]wk of PA had an odds ratio of LLL of 0.32 (95% CI: 0.15-0.69; Ptrend = .003). Stratified analyses suggested the association of PA and LLL existed only among women with a body mass index (BMI) <30 kg/m (Ptrend = .007), compared to women with a BMI ≥30 kg/m (Ptrend = .47). Compared with participants who reported <4.0 blocks[BULLET OPERATOR]d of walking, participants who reported ≥12 blocks[BULLET OPERATOR]d of walking had an odds ratio of LLL of 0.19 (95% CI: 0.09-0.43; Ptrend < .0001). Stratified analyses suggested the association of walking and LLL was similar among women with a BMI <30 kg/m (Ptrend = .007) and women with a BMI ≥30 kg/m (Ptrend = .03).

CONCLUSION:

Participation in higher levels of PA or walking is associated with reduced proportions of LLL in dose-response fashion. These findings should be interpreted as preliminary, and investigated in future studies.