Lymphedema Research Articles

VARIOUS CLINICAL SCENARIOS IN SECONDARY MALIGNANT LYMPHEDEMA

Diagnosing malignant lymphedema is a challenge in daily clinical practice. Clinically, patients may show clear signs of malignancy, but this is not always the case, and at times the diagnosis is not straightforward. In some patients, pain, hardness of the tissues, joint stiffness, proximal involvement, collateral circulation, or an acute onset will provide the clue to determining malignancy. Our aim is to describe several diverse scenarios of Secondary Malignant Lymphedema (SML) with the etiopathogenesis. One possible cause is lymphatic obstruction due to extrinsic compression of lymphatic vessels and/or nodes by either the primary tumor or metastatic masses. Lymphatic obstruction can also be caused by tumoral infiltration. This infiltration can affect both deep and regional nodes as well as cutaneous and subcutaneous vessels and is commonly known as lymphangitis carcinomatosa. Malignant lymphedema can also be secondary to obstruction of the venous flow due to tumoral venous thromboembolism or to extrinsic compression of the veins by tumors or adenopathic masses. Nevertheless, the most frequent cause of this illness is a mixed mechanism of compression of the lymphatic and venous systems. Frequently, SML is the first manifestation of relapse. When lymphedema appears abruptly, is progressive, with intense pain, associated with collateral circulation, or with hard and infiltrated skin or joint stiffness, SML must be ruled out with an urgent referral to the oncologist and an imaging evaluation.

Thromboxane prostanoid signaling in macrophages attenuates lymphedema and facilitates lymphangiogenesis in mice : TP signaling and lymphangiogenesis.

Accumulating evidence suggests that prostaglandin E2, an arachidonic acid (AA) metabolite, enhances lymphangiogenesis in response to inflammation. However, thromboxane A2 (TXA2), another AA metabolite, is not well known. Thus, this study aimed to determine the role of thromboxane prostanoid (TP) signaling in lymphangiogenesis in secondary lymphedema. Lymphedema was induced by the ablation of lymphatic vessels in mouse tails. Compared with wild-type mice, tail lymphedema in Tp-deficient mice was enhanced, which was associated with suppressed lymphangiogenesis as indicated by decreased lymphatic vessel area and pro-lymphangiogenesis-stimulating factors. Numerous macrophages were found in the tail tissues of Tp-deficient mice. Furthermore, the deletion of TP in macrophages increased tail edema and decreased lymphangiogenesis and pro-lymphangiogenic cytokines, which was accompanied by increased numbers of macrophages and gene expression related to a pro-inflammatory macrophage phenotype in tail tissues. In vivo microscopic studies revealed fluorescent dye leakage in the lymphatic vessels in the wounded tissues. The results suggest that TP signaling in macrophages promotes lymphangiogenesis and prevents tail lymphedema. TP signaling may be a therapeutic target for improving lymphedema-related symptoms by enhancing lymphangiogenesis.

Association of clinical manifestations of secondary lymphedema and lymph node dissection sites in the lower extremities of patients with melanoma

Background Lymphedema is a chronic, debilitating disease that often requires life-long management. Predicting clinical manifestations and prognosis is crucial in clinical practice because the treatment of lymphedema should be individualized for best clinical outcome. The aim of this study is to explore the location and severity of lymphedema secondary to inguinal and/or iliac lymph node dissection (LND) in patients with melanoma. Methods Patients with melanoma who received LND at a single tertiary medical center between 1 January 2010 and 31 September 2022 were retrospectively reviewed. Patient who received inguinal LND only were designate as the inguinal group while those who received both ilioinguinal LND were included in the ilioinguinal group. Volumetric measurement was used to objectify the severity and location of lymphedema. Clinical data was acquired for 12–15 months of follow-up. Results Among 81 patients, 43 (53%) had developed lymphedema in the lower extremities at an average of 33 days after the surgery. Initially, patients manifested with medial thigh lymphedema in the inguinal group while patients were presented with whole leg lymphedema in the ilioinguinal group. Lower leg volume of the ilioinguinal group was significantly higher than the inguinal group. After more than 12 months of lymphedema treatment, upper leg volume was higher in the ilioinguinal group than the inguinal group (12.7% vs 5.4%, p < 0.05). Conclusion Lymphedema developed in early post-op period. The ilioinguinal group presented with a larger volume of lymphedema in the distal area of the legs. Even after sufficient treatment, predominant lymphedema remained in the proximal leg for the ilioinguinal group. Patients with both inguinal and iliac LND were associated with more severe lymphedema. Based on the dissection sites, the clinical manifestations and prognosis of leg lymphedema can vary widely. Thus, clinicians should consider the dissection site when approaching melanoma patients with lymphedema.

The Utilization of e-Health in Lymphedema Care: A Narrative Review.

Background: Electronic health (e-Health), refers to technologies that can be utilized to enhance patient care as well as collect and share health information. e-Health comprises several umbrella terms, including telehealth, mobile health, e-Health, wearables, and artificial intelligence. The types of e-Health technologies being utilized in lymphedema (LE) care are unknown. Method: In this narrative review, a search of published research on the utilization of e-Health technologies in LE-related care was conducted. Results: Five different types of e-Health modalities were found (robotics, artificial intelligence, electronic medical records, smart wearable devices, and instructive online information) spanning 14 use cases and 4 phases of care (preventative, diagnostic, assessment, and treatment phases). Broad e-Health utilization examples were found including robotic-assisted surgery to reduce the likelihood of LE after lymphadenectomy, machine learning to predict patients at risk of filarial-related LE, and a novel wearable device prototype designed to provide lymphatic drainage. Conclusions: e-Health has reported merit in the prevention, diagnoses, assessment, and treatment of LE with utilization demonstrating cutting edge applicability of e-Health for achieving optimal patient care and outcomes. As technology continues to advance, additional research into the utilization of e-Health in LE care is warranted.

Changes in intracellular water volume after leg lymphedema onset and lymphaticovenular anastomosis as its surgical intervention

ObjectiveTo clarify the changes in the intracellular water (ICW) volume in lymphedema-affected legs after lymphedema onset and its surgical intervention (ie, lymphaticovenular anastomosis [LVA]), we investigated the changes in body water composition using bioelectrical impedance analysis. MethodsThis retrospective case series included 41 women with unilateral secondary leg lymphedema. The volume changes in the ICW and extracellular water (ECW) of the affected leg were measured using an InBody S10 (InBody Co, Ltd) multifrequency bioelectrical impedance analyzer, at both lymphedema onset and 1 year after LVA. ResultsThe volume increase with leg lymphedema onset was comparable between the ECW and ICW (0.59 L vs 0.56 L; 95% confidence interval [CI], −0.02 to 0.06; P = .27), and the increase rate was higher for ECW (35.3% vs 22.1%; 95% CI, 9.3%-17.2%; P < .001). The volume reduction at 1 year after LVA was comparable between ECW and ICW (0.23 L vs 0.27 L; 95% CI, −0.08 to 0.02; P = .20), and the reduction rate was higher for ECW (8.7% vs 7.0%, 95% CI, 0.04%-3.2%; P = .044). The volume difference between ICW and ECW remained constant throughout the six measurements before and after LVA (F[3.01, 120.20] = 1.85; P < .14). ConclusionsLeg LVA reduced ICW in the lymphedematous leg. The onset of leg lymphedema increased ECW and ICW in the affected limb, and LVA decreased both ECW and ICW. The volume change in the affected leg was comparable between ECW and ICW at both lymphedema onset and after LVA. However, the rate of change was higher for ECW. The volume difference between ICW and ECW remained constant. Using bioelectrical impedance analysis, alterations in ICW volume were detected in the legs affected by lymphedema, both after the onset of lymphedema and after LVA intervention.

Concomitant Venous Thrombosis in Cellulitis of the Upper Extremity

Abstract Objectives Concurrent thrombosis is observed in up to 15 % of the patients with cellulitis of the lower extremity. The risk of concomitant venous thrombosis in patients with upper extremity cellulitis has not been investigated, yet. Aim of this study is to examine the frequency of concomitant venous thrombosis in patients with cellulitis of the upper extremity and to identify risk factors, comorbidities, and complications. Materials and Methods From 2014 to 2021, patients with cellulitis of the arm that received duplexsonography of the same limb were included. Files were reviewed for demographic data, comorbidities, and risk factors. Results 53 patients with upper limb cellulitis were included. In 11.3 % of the patients (n = 6), concomitant venous thrombosis of the upper extremity was detected. Three patients had deep arm thrombosis (brachial and subclavian veins), three had superficial vein thrombosis. Conclusion Cellulitis may increase the risk for venous thrombosis. Venous catheters and chronic lymphedema favor the occurrence of both cellulitis and venous thrombosis. Oncological patients are at particular risk for developing concomitant venous thrombosis.

Compression wraps as adjuvant therapy in the management of acute systolic heart failure

BackgroundGuidelines recommend targeting decongestion in management of decompensated HF, with lower extremity edema often serving as the clinical target. LECW are seldom used in the acute setting, with a paucity of data on efficacy in HF, despite serving as a cornerstone of chronic lymphedema management. Primary objectiveStudy the efficacy and safety of LECW in acute decompensated HF. MethodsOpen-label, randomized, parallel-group clinical trial. Primary outcomesDays on intravenous (IV) diuretic therapy, total hospital length of stay (LOS), and 30-day all-cause readmission. Results32 patients were enrolled, with 29 patients completing the study. Enrollment was suspended due to the COVID-19 pandemic. Overall LOS was shorter in the intervention group (3.5 vs 6 days, p = 0.05), with no significant difference in total days on IV diuresis or 30-day readmission rate with use of LECW. Fewer patients required continuous diuretic infusion after treatment with LECW (0 vs 7 patients, p = 0.027). The intervention group scored significantly better on the MLWHF (55.5 vs 65, p = 0.021), including both the physical and emotional dimension scores. No adverse events were reported with use of LECW, including a significantly lower incidence of AKI (1 vs 13 patients, p = 0.005). ConclusionThe use of LECW resulted in reduced hospital LOS compared to standard therapy, with no difference in days of IV diuresis administration or 30-day readmission. Treatment with LECW also resulted in less continuous IV diuretic therapy, fewer incidence of AKI, and improved quality of life. Trends toward less escalation of diuresis, and greater reduction in edema were also observed.

Substance P promote macrophage M2 polarization to attenuate secondary lymphedema by regulating NF-kB/NLRP3 signaling pathway

Secondary lymphedema often occurs after filariasis, trauma, lymph node dissection and radiation therapy, which is manifested by infiltration of inflammatory cells and fibrosis formation in pathologically. Substance P is a widely used neuropeptide in the field of tissue repair, while the regenerative potential of the substance P has not been proven in the secondary lymphedema. In this study, animal model of secondary lymphedema was constructed by excising the skin and subcutaneous lymphatic network in the tail of mice, and the degree of swelling in the tail of mice was evaluated after 6 weeks under the treatment with substance P. Immunofluorescence staining was also performed to assess immune cell infiltration, subcutaneous fibrosis and lymphangiogenesis. The results revealed that substance P significantly alleviated post-surgical lymphedema in mice. Furthermore, we found that substance P promoted macrophages M2 polarization, a process associated with downregulation of the NF-kB/NLRP3 pathway. After application of disodium clodronate (macrophage scavenger, CLO), the positive effect of substance P in lymphedema is significantly inhibited. In vitro experiments, we further demonstrated the polarizing effect of substance P on bone marrow-derived macrophages (BMDMs), while substance P inhibited the activation of the NF-kB/NLRP3 pathway in BMDMs after the treatment of lipopolysaccharide (LPS). In addition, polarized macrophages were demonstrated to promote the proliferation, tube-forming and migratory functions of human lymphatic endothelial cells (hLEC). In conclusion, our study provides preliminary evidence that substance P alleviates secondary lymphedema by promoting macrophage M2 polarization, and this therapeutic effect may be associated with downregulation of the NF-kB/NLRP3 pathway.

Treatment with YIGSR peptide ameliorates mouse tail lymphedema by 67 kDa laminin receptor (67LR)-dependent cell-cell adhesion

Impaired microcirculation can cause lymphatic leakage which leads to a chronic swelling in the tissues of the body. However, no successful treatment gives any protection against lymphedema due to the lack of well-revealed pathophysiology of secondary lymphedema. Binary image of laminin immunohistochemical expression revealed that distribution of laminin expression localized during surgically induced lymphedema. 67 kDa laminin receptor (67LR) mRNA expression showed a peak at during lymphedema exacerbation. Since the response of 67LR molecules may affect the prevention of inflammation and edema, here we have hypothesized that 67LR ligand of YIGSR peptide could permit reconstructive environment for amelioration of lymphedema and evaluated the effect of YIGSR in a mouse tail model of lymphedema. Indeed, intra-abdominal injections of YIGSR for the first 3 days after inducing lymphedema in the mouse tail model reduced the tail lymphedema on day 14 by 27% (P = 0.035). Histology showed that YIGSR treatment protected lymphedema impairment in epidermis and dermis, and it also inhibited the expansion of intercellular spaces and enhanced especially cell adhesion in the basement membrane as revealed by transmission electron microscopy. Interestingly, the treatment also reduced the local expression of transforming growth factor (TGF)β. Further elucidation of the mechanisms of 67LR-facilitated lymphangiogenesis contributes to find potential targets for the treatment of lymphedema.

Experimental Study of the Prevention and Treatment of Lymphoedema in a Rat Hindlimb Model by Applying Cardioperitoneal Catheters and Lacrimal Intubation Tubes in the Inguinoperitoneal Region

Secondary lymphoedema (LE) is a chronic condition with limited surgical treatment options for restoring extremity form and function. This study aims to establish a reproducible model of secondary LE and evaluate the preventive and corrective effects of fenestrated catheters (FC) and capillary tubes (CT). A total of 35 rats underwent left hindlimb inguinal and popliteal node dissection, followed by radiotherapy after two weeks. The right hindlimb served as the control. Rats were divided into five groups: sham, two preventive (Group 2 - EFC, Group 3 - ECT), and two corrective (Group 4 - LFC, Group 5 - LCT). Measurements of ankle circumference (AC) and paw thickness (PT) were taken weekly, and imaging modalities were performed. After a 16 week follow up, rats were euthanised for histological examination. Data include paw thickness (PT) and ankle circumference (AC) ratios for hindlimbs. In the sham group, AC ratio was 1.08 (p = .002) and PT ratio was 1.11 (p = .020), confirming successful lymphoedema model establishment. Early catheter and tube placement in Groups 2 and 3 prevented AC and PT increase until the 16th week. Group 2: AC ratio was 0.98 (p = .93), PT ratio was 0.98 (p = .61). Group 3: AC ratio was 0.98 (p = .94) and PT ratio was 0.99 (p = .11). From the 10th to the 16th week, Groups 4 and 5 exhibited reduced measurements after insertion of catheters and tubes. Computed tomography imaging as an objective examination supported the results obtained from the measurements. The histological findings confirmed the benefits of both FC and CT. The insights gained from the present study provide a basis for further exploration and refinement of drainage system designs, ultimately leading to improved treatment approaches for individuals suffering from lymphoedema in the future.

Development of a rat model of lymphedema and the implantation of a collagen-based medical device for therapeutic intervention.

Secondary lymphedema is a common condition among cancer survivors, and treatment strategies to prevent or treat lymphedema are in high demand. The development of novel strategies to diagnose or treat lymphedema would benefit from a robust experimental animal model of secondary lymphedema. The purpose of this methods paper is to describe and summarize our experience in developing and characterizing a rat hindlimb model of lymphedema. Here we describe a protocol to induce secondary lymphedema that takes advantage of micro computed tomography imaging for limb volume measurements and visualization of lymph drainage with near infrared imaging. To demonstrate the utility of this preclinical model for studying the therapeutic benefit of novel devices, we apply this animal model to test the efficacy of a biomaterials-based implantable medical device.

Therapeutic effect of microcurrent therapy in a rat model of secondary lymphedema.

Secondary lymphedema is a clinically incurable disease that commonly occurs following surgical cancer treatment and/or radiation. Microcurrent therapy (MT) has been shown to decrease inflammation and promote wound healing. This study aimed to investigate the therapeutic effect of MT in a rat model for forelimb lymphedema induced by axillary lymph node dissection. The model was created by dissecting the right axillary lymph node. Two weeks after surgery, 12 Sprague-Dawley rats were randomly divided into two groups: one that underwent MT in the lymphedematous forelimb (MT, n=6) and a sham MT group (sham MT, n=6). MT was applied daily for 1 h in each session for two weeks. The circumferences of the wrist and 2.5 cm above the wrist were measured 3 days and 14 days after surgery, weekly during MT and 14 days after the last MT. Immunohistochemical staining of pan-endothelial marker (CD31), Masson's trichrome, and western blot analysis of vascular endothelial growth factor C (VEGF-C) and vascular endothelial growth factor receptor-3 (VEGFR3) were performed 14 days after the last MT. Quantification of the area covered by blood vessels (CD31+) and fibrotic tissue area were measured using an image analysis program (ImageJ software). The circumference of the carpal joint in the MT group was significantly decreased 14 days after the last MT compared to that in the sham MT group (P=0.021). The area covered by blood vessels (CD31+) was significantly higher in the MT group than in the sham MT and contralateral control group (P<0.05). The extent of fibrotic tissue was significantly attenuated in the MT group compared to the sham MT group (P<0.05). The expression of VEFGR3 was 2.02-fold higher for MT group, compared for the contralateral control group, which was statistically significant (P=0.035). VEGF-C expression was 2.27-fold higher for MT group than that for contralateral control group; however, the difference between the groups was not significant (P=0.051). Our findings indicate that MT promotes angiogenesis, and improves fibrosis in secondary lymphedema. Therefore, MT may be a novel and non-invasive treatment modality for secondary lymphedema.

Multi-segment bioimpedance in the evaluation of treatment for secondary lower limb lymphedema: Gynecological cancer

&lt;b&gt;Aim&lt;/b&gt;: The present aimed study was to report the mobilization of body fluids after five days of intensive treatment for secondary lower limb lymphedema.&lt;br /&gt; &lt;b&gt;Materials and methods&lt;/b&gt;: A crossover clinical trial was conducted involving the analysis of changes in intracellular and extracellular fluids in all extremities and the trunk in women with lower limb lymphedema secondary to treatment for gynecological cancer. Evaluations were performed with multi-segment bio impedance analysis before and after intensive treatment using Godoy Method® eight hours per day for five days. Comparisons were made using Wilcoxon signed-ranks test.&lt;br /&gt; &lt;b&gt;Results&lt;/b&gt;: Significant changes in intracellular and extracellular fluids were found in all extremities and the trunk (p&amp;lt;0.0001 for all comparisons).&lt;br /&gt; &lt;b&gt;Conclusion&lt;/b&gt;: Intensive Godoy Method® leads to important changes in the distribution of body fluids in the treatment of lower limb lymphedema following treatment for gynecological cancer, with a significant increase in fluids in the upper limbs and trunk as well as significant reductions in the lower limb and in intracellular and extracellular water.

Components of the fascia – cells and extracellular matrix

Abstract Fascia continues to be a significant topic in numerous studies due to its unique functions that contribute to maintaining various bodily functions. The current article focuses on the crucial aspects of fascia and provides an essential collection of basics to understand the phenomenon of fascia. It describes the anatomical structure, with emphasis on the division into layers. Furthermore, the article explains how fascia affects muscle movement and transduction signals. In addition to the significant role of fascia, the article discusses its components, both cellular and non-cellular. Regarding cellular factors, various types of cells are distinguished and their role in mechanotransduction is explained. Non-cellular components maintain the structure of the fascia and provide scaffolding for the cells of the fascia. The problem of fascia classification is comprehensively considered, including the anatomical, histological, and cellular aspects of fascia. Finally, the article examines aspects of fascia disorders, particularly rheumatoid arthritis, as well as the correlation of structural abnormalities with lymphatic oedema. Not only does it address these abnormalities, but it also discusses the nature of myofascial pain origin and the currently widespread topic of acupuncture.

Effectiveness of physical exercise programmes in reducing complications associated with secondary lymphoedema to breast cancer: a protocol for an overview of systematic reviews

IntroductionBreast cancer-related lymphoedema (BCRL) is one of the most underestimated and debilitating complications associated with the treatment that women with breast cancer receive. Several systematic reviews (SRs) of different physical...

Factors That Influence Compliance with Self-Care Management of Chronic Lymphedema: A Scoping Review

Abstract Date Presented 04/21/2023 Lymphedema is an ongoing disease that requires daily self-management to control symptoms and decrease the risk of progression. Patient adherence with self management programs is an important part of daily maintenance. Understanding the factors that affect patient adherence will allow clinicians to improve outcomes and quality of life for their clients. Primary Author and Speaker: Patricia Watford Additional Authors and Speakers: Caroline Corso, Jamie Freeman, Mia Marrow, Melinda Coody, Montana Moreno, Alexis Perkins

Obesity and secondary lymphedema

Obesity causes all types of lymphedema to deteriorate. Obesity-associated lymphedema is by now the most frequent secondary lymphedema and constitutes an entity in its own right. Obesity and its comorbidities, due to mechanical and inflammatory effects, decrease lymphatic transport and create avicious circle of lymph stasis, local adipogenesis, and fibrosis. The therapeutic strategy must therefore address both lymphedema and obesity and its comorbidities.

Surgical management of acquired buried penis and scrotal lymphedema: A retrospective review

Acquired buried penis is a condition that can have detrimental physical and psychological consequences for patients. Factors such as elevated BMI, chronic scrotal lymphedema, hidradenitis suppurativa, and chronic inflammation can lead to the condition. Surgical intervention is the treatment of choice for advanced disease. Following IRB approval, a retrospective chart review was performed for patients with a diagnosis of acquired buried penis who required surgical intervention. Details of patient history, surgical management including intraoperative and post-operative photography, and complications were reviewed. Seven patient cases were reviewed. The average age at time of surgery was 44 with a mean weight of 344 pounds and an average BMI of 48. Severe scrotal lymphedema and hidradenitis were common concurrent comorbidities. Concurrent scrotoplasty and infraumbilical panniculectomy were standard parts of the operations. Native glans skin was salvageable in all but one case. Penile shaft skin was reconstructed with skin grafts or adjacent tissue transfer. 88% of the cases had some element of wound dehiscence post-operatively. Surgical management of an acquired buried penis can be challenging. The patient demographic with the disease is frequently complicated by morbid obesity, concurrent lymphedema, or hidradenitis. Post-operative complications are expected. The surgical techniques presented can aid in simplifying the management of this challenging surgical population.

Risk of Lymphedema and Death after Lymph Node Dissection with Neoadjuvant and Adjuvant Treatments in Patients with Breast Cancer: An Eight-Year Nationwide Cohort Study.

Knowledge on the impact of neoadjuvant and adjuvant treatments on post-surgery lymphedema (LE) in patients with breast cancer is limited due to methodological limitations and an insufficient sample size. We investigated the risk of LE in patients going through long-term anticancer treatment regimens using a national cohort from the Korean National Health Insurance Service database from 2011-2013. Incidence rate ratio, Kaplan-Meier analysis, and Cox proportional regression analysis were performed. A total of 39,791 patients were included. While minimal lymph node dissection (SLNB) reduced the risk of LE (hazard ratio [HR] 0.51) as expected, neoadjuvant chemotherapy (NAC) followed by SLNB did not reduce the risk. Adjusting for adjuvant chemotherapy (AC) as time-varying exposure decreased the risk of LE in the SLNB group (HR 0.51), but not the mortality risk (HR 0.861). A longer duration of NAC, especially taxane-based, combined with SLNB reversed the effect and increased risk of LE. The findings highlight the importance of not only early surveillance before and after surgery, but also long-term surveillance during adjuvant treatment by surgeons and oncologists in order to reduce the risk of LE.

Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report

BackgroundComposite haemangioendothelioma is a rare vascular neoplasm with indolent to intermediate malignant potential. Diagnosis of this disease relays on histopathological identification of at least two different morphologically distinctive vascular components in proper clinical settings. Exceedingly rare cases of this neoplasm can exhibit areas resembling high-grade angiosarcoma, which does not change the biological behaviour. Such lesions tend to occur in the setting of chronic lymphoedema and thus, can mimic Stewart-Treves syndrome, which has a much worse clinical outcome and prognosis.Case presentationWe present a case of 49 years old male suffering from chronic lymphoedema of the left lower extremity who had developed a composite haemangioendothelioma with high grade angiosarcoma-like areas mimicking the Stewart-Treves syndrome. Given the multifocality of the disease, the only potentially curable surgical treatment would be hemipelvectomy, which was refused by the patient. The patient has been followed-up, with no signs of local progression of the remaining disease, nor a distant spread outside the involved extremity for two years.ConclusionsComposite haemangioendothelioma represents a rare malignant vascular tumour, with significantly more favourable biological behaviour than angiosarcoma, even in cases where angiosarcoma-like areas are present. For that reason, composite haemangioendothelioma can be easily misdiagnosed as true angiosarcoma. The rarity of this disease unfortunately hampers the development of clinical practice guidelines and the implementation of treatment recommendations. Most of the patients with localized tumour are treated by wide surgical resection, without neo- or adjuvant radiotherapy or chemotherapy. However, in the case of this diagnosis, the watch-and-wait approach is better than mutilating procedure, highlighting the necessity of establishing of the correct diagnosis.