Breast cancer-related lymphedema (BCRL) affects approximately 20% of women after breast cancer therapy. Advances in treatment have increased life expectancy, thus the prevalence of BCRL will continue to rise with the number of cancer survivors. Hence the need to develop strategies to prevent this condition. We provide a systematic review of the literature on primary prevention of BCRL by prophylactic lymphatic surgery (PLS). Between June and August 2022, we conducted a search of PubMed, Google Scholar and Cochrane. In the end a total of eighteen papers were selected. The eleven studies without a control group reported only 15 of 342 patients who developed lymphedema at least six months after PLS (4.59%). The seven studies with a control group included 569 patients, 328 cases and 241 controls. Among the cases, 36 (10%) developed lymphedema. In contrast, the incidence of lymphedema in controls was 40% (98 of 241 patients). Formulation of definite recommendations in favour of PLS is hindered by low-quality studies. There is no consensus on which technique should be preferred, nor on whether adjuvant radiotherapy might affect the efficacy of PLS. Randomized controlled trials are mandatory to conceive evidence-based recommendations.

Lymphedema refers to a group of disorders characterized by the accumulation of protein-rich fluid in the interstitial tissues, resulting from an imbalance between lymph production and it’s drainage via lymphatic vessels. More than 10 types of isolated primary LD is described, and among them CELSR1-associated lymphedema are one of the less studied. A family with two sibs, a 17-year-old boy and an 8-year-old girl, both diagnosed with primary LD, presented to the clinic. Both patients were observed with primary symptoms of LD at 2-3 months of age, presented by the swelling of right foot. Lymphatic pathologies were not diagnosed in parents or close relatives. The rare c.7664A>C variant, resulting in the H2555P missense mutation in the intracellular loop of CELSR1 transmembrane domain, was discovered in a patients. The clinical signs, MRL scanning data, in silico predictors and the absence of known pathogenic mutations or rare variants of unknown significance in other genes, associated with the development of primary lymphedema, allow us to assume that c.7664A>C variant of CELSR1 may be responsible for pathogenesis of the disease in the presented family case.

Milroy's disease (MD), also known as hereditary lymphedema type I, is a rare autosomal dominantly inherited primary lymphedema. It accounts for approximately twenty percent of all primary lymphedemas. MD typically presents clinically with bilateral lower extremity lymphedema. Our case report is about a rare case of MD presenting with unilateral lymphedema in both upper and lower extremities in a patient admitted to our clinic. A 24-year-old woman who had been under observation for lymphedema since she was 8 years old was diagnosed with MD by physical examination, venous Doppler ultrasound, whole body lymphoscintigraphy, and molecular genetic testing in our clinic, and partial improvement was achieved with Complete Decongestive Therapy, the gold standard treatment for lymphedema. Genetic counseling services may be especially useful for the patient and family. Although MD is rarely seen in the literature, many more studies are needed to identify carrier variants in the community.

Abstract: Exercise has been repeatedly shown to be safe and beneficial for cancer survivors. However, there is no normative guideline for exercise prescription, and it is under exploration. Therefore, this literature review aims to provide some advice for the formulation of exercise prescriptions for patients with breast cancer-related lymphedema (BCRL) from the perspective of reducing lymphedema severity. A review of relevant studies published before November 2023 was conducted using three scientific databases PubMed, Embase, and Scopus. A total of 2696 articles were found. Eventually, 13 studies fulfilled the inclusion criteria and were included in this literature review. We concluded daily, or nearly daily exercise at home can be recommended. Moreover, reduced lymphedema severity may not be maintained after ceasing the exercise program, so exercise should be a lifelong practice.

Under inflammatory conditions including lymphatic disorders, bone marrow-derived myeloid cells often express lymphatic endothelial cell (LEC) markers called LEC progenitor cells, which extend lymphatic vessels by fusing with existing lymphatic vessels. However, studies on the mechanism of lymphatic regeneration using three-dimensional images of lymphatic structures are limited. In this study, scanning electron microscopy (SEM) was used to observe the three-dimensional structure of lymphangiogenesis in a mouse model of secondary lymphedema. The model was established in C57BL/6J mice via circumferential incision in the inguinal region of the left hind limb. Skin samples were obtained from the lymphedema region on days 2, 5, and 8 after surgery. To determine lymphatic vessel positions using SEM analysis, we detected anti-lymphatic vessel endothelial hyaluronan receptor 1 (LYVE-1) immunoreactivity in serial sections and overlaid them during SEM observation. On days 2 and 5, spherical cells, probably myeloid cells, were attached and fused to the LYVE-1-positive lymphatic vessel walls. On day 8, spherical cells were converted to string-shaped cells, forming a new lymphatic vessel wall resembling an intraluminal pillar. Our results show the three-dimensional lymphatic structures formed during lymphatic regeneration.

Lymphedema is a chronic and debilitating disease that affects up to 250 million patients worldwide. Recent advances in understanding its pathophysiology, along with improved diagnosis and microsurgical techniques, have enhanced our ability to cope with the challenging task of treating this disease. This review provides an overview of the disease from a surgeon's point of view, including existing imaging modalities used for preoperative assessment, as well as surgical procedures used in its treatment. Advantages and drawbacks of various existing modalities used for pre or intraoperative assessment of lymphatic vessels are discussed. Lymphedema treatment has shifted from palliative debulking procedures (liposuction and direct excision) to those aimed at restoring lymphatic flow and countering the pathophysiology of the disease (lymphaticovenous anastomosis and vascularized lymph node transfer). A combination of both approaches can result in a synergistic benefit for patients and are discussed in this review. Despite recent advances, some controversies persist, and further studies are needed to better define surgical treatment algorithms.

(1) Background: Breast cancer-associated lymphedema (BCRL) is a major complication of breast cancer treatment, affects the quality of life of breast cancer survivors who develop it. Manual lymphatic drainage has a beneficial result in combating lymphedema in patients who have undergone breast cancer surgery. The hypothesis consists in complete decongestive therapy reduces the lymphedema at the level of the upper limb on the side of mastectomy. (2) Methods: Twenty-five patients with radical mastectomy and upper limb lymphedema on the mastectomy side were included in this study. Upper limb circumferences were measured with a tape measure at the following levels: metacarpophalangeal joint; distal radioulnar joint; 10 cm distal to lateral epicondyle; 5 cm proximal to elbow joint; 10 cm proximal to lateral epicondyle. (3) Results: Patients received twice weekly manual lymphatic drainage, multi-layer compression bandaging and upper limb muscle stimulation exercises on the mastectomy side twice weekly. The circumference assessed at the level of the metacarpophalangeal joints and distal radio-carpal joint decreased at the final evaluation. Forearm perimetery - 5 and 10 cm distal to the lateral epicondyle the circumference decreased at the final assessment. (4) Conclusions: Patients with breast cancer-related lymphedema should receive comprehensive decongestive therapy in the early stage of lymphedema to achieve rapid clinical results and improved quality of life

Background: Primary lymphedema is a chronic condition caused by a developmental abnormality of the lymphatic system and its malfunction. Different surgical options such as physiologic and excisional procedures have been proposed. The aim of this study was to present a complete algorithm for the treatment of primary lower extremity lymphedema: Primary LYmphedema Multidisciplinary Approach (P-LYMA). Methods: Nineteen patients were treated according to the P-LYMA. Patients underwent pre- and post-operative complex decongestive therapy (CDT). Different physiologic and excisional procedures were performed independently or in combination. The primary outcome was the circumferential reduction rate (CRR). The Lymphedema Quality of Life Score (LeQOLiS), reduction in the number of episodes of cellulitis, length of stay, and complications were recorded. Results: The mean CRR calculated was 72.8±19.6% after twelve months. The episodes of cellulitis per year decreased from a mean of 1.9±0.8 preoperative to 0.4± 0.6 throughtout follow-up. Two patients experienced minor complications. Mean hospitalization time was 5 days. Patients’ quality of life (LeQOLi score) drastically improved from 70.4±11.6 to 24.2 ±13.9 at twelve months post-op. Conclusion: The P-LYMA algorithm allows to maximize surgical results and improve the quality-of life in patients who suffer from primary lymphedema. CDT is mandatory to optimize results.