RESUMEN

Although a low-grade vascular tumor, Kaposi sarcoma (KS) can have mucosal, and visceral involvement. Additionally, disfiguring disseminated lesions can be seen in patients with human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS). KS may cause lymphatic obstruction leading to chronic lymphedema that further contributes to progressive cutaneous hypertrophy and severe disfigurement in the form of non-filarial elephantiasis nostras verrucosa (ENV). This report highlights a case of a 33-year-old male with AIDS who presented in acute respiratory distress with bilateral lower extremity nodular lesions. We confirmed a diagnosis of KS with overlying ENV via a multi-disciplinary approach. Collaboratively, we optimized our patient and observed adequate treatment response and overall improvement in clinical status. Our report emphasizes the importance of a multi-disciplinary approach in recognizing a rare presentation of ENV. Recognition of the disease and understanding the extent of the disease are crucial in preventing irreversible disease progression and allowing for maximum response.

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Chronic lymphedema is fraught with morbidity, including tissue loss. We present the case of a woman with long-standing lymphedema suffering from nonhealing ulcerations despite multiple interventions, who underwent below-knee amputation. Surgical pathology yielded a diagnosis of invasive squamous cell carcinoma. We highlight the uncommon association between lymphedema and squamous cell carcinoma, and the importance of routine pathological testing with lower extremity amputations.

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PURPOSE: We investigated whether a one-stage combination of vascularized lymph node transfer (VLNT) with water jet-assisted liposuction (WAL) can be safely performed and results in improved patient outcomes such as a greater reduction in arm volume when treating chronic breast cancer-related lymphedema (BCRL). METHODS: In this retrospective cohort study, we included all patients from our encrypted lymphedema database treated for chronic BCRL with VLNT or VLNT + WAL who had a minimum follow-up of two years. We analyzed patient-specific variables including arm circumferences as well as patient-reported outcomes before and after surgery as well as surgery time, surgery-related complications and patient satisfaction. RESULTS: Only the mean preoperative differences of the circumferences between the lymphedematous and the unaffected arm in individual patients showed a statistically significant difference between treatment groups (p < 0.05). Indeed, patients treated with VLNT + WAL had consistently larger differences in individual sets of arms and therefore more pronounced chronic BCRL. The mean surgery time was significantly longer in the VLNT + WAL group (p < 0.05). Complications were seldom and similar in both groups. Using a numeric rating scale, the level of patient satisfaction following treatment did not differ significantly between groups (p = 0.323). CONCLUSIONS: Our findings suggest that a one-stage combination of VLNT with WAL does not result in more complications even though it also entails a longer surgery time. This is acceptable as secondary interventions resulting in overall longer surgery times and higher costs can be avoided. A one-stage combination might be especially favourable for patients suffering from more severe chronic BCRL.

Asunto(s)

Linfedema del Cáncer de Mama , Neoplasias de la Mama , Linfedema , Humanos , Femenino , Estudios Retrospectivos , Neoplasias de la Mama/complicaciones , Neoplasias de la Mama/cirugía , Linfedema del Cáncer de Mama/etiología , Linfedema del Cáncer de Mama/cirugía , Linfedema/etiología , Linfedema/cirugía , Brazo , Ganglios Linfáticos

RESUMEN

Background: Despite the lymphatic system being so important and extensive, the field of lymphatic diseases, research is still very young. Lymphedema is a progressively debilitating condition with no known "cure." Specific pathologies that could benefit from improved lymphatic drainage by advanced super surgical techniques or engineered tissue transfer are being sought. Microsurgical techniques like lymphovenous bypass and anastomosis have spurred interest as they tend to physiologically restore the damaged lymphatic channels and may be a key to permanent cure. The latest in the field is vascularized lymph node transfer (VLNT), indicated in post mastectomy or other post operative settings producing disruption of regional lymphatic channels and draining lymph nodes. Autologous healthy lymph nodes are transferred along with surrounding fat and vascular pedicle to the affected limb in a bid to promote lymphangiogenesis. Lymphoscintigraphy (LS) is a simple, noninvasive nuclear technique used in identifying upper or lower limb lymphatic dysfunction and obstruction with a high degree of sensitivity. Quantitative LS is extremely useful in follow-up assessment of lymphedema postmanual lymphatic drainage (MLD) or other forms of medical management. Aim: We hypothesize that LS can document perinodal lymphangiogenesis post VLNT. Material and Methods: Three cases of acquired lymphedema (suspected filariasis and postmastectomy conditions) who underwent VLNT in our institute were prospectively studied with LS. The imaging findings highlight the subtle lymphatic regeneration along with the vascularized graft in all three patients during the early postoperative period. Conclusion: This is the first (pilot) study documenting early spontaneous perinodal lymphangiogenesis after VLNT in human subjects.99mTc Nanocolloid LS has been found to be incremental in demonstrating early lymphangiogenesis.

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OBJECTIVE: To examine acceptance of disability, coping style, perceived social support, and quality of life and to explore the relationships between acceptance of disability, coping style, perceived social support, and quality of life among Chinese patients with chronic lymphedema. METHODS: Chronic lymphedema patients were recruited from five tertiary hospitals between May and July 2020 in China. Recruited patients were assessed for quality of life (QOL), acceptance of disability (AOD), coping styles, perceived social support (PSS), and sociodemographic and disease-related factors. Multivariate linear regression models were conducted to examine the multivariate effect of AOD, coping style, PSS, and sociodemographic and disease-related factors on QOL. RESULTS: A total of 163 chronic lymphedema patients were recruited. The mean score of QOL was 2.23 (SD = 0.68). AOD, number of symptoms, acceptance-resignation, avoidance, degree of pain, PSS, and educational level were found to be significant predictors of QOL. CONCLUSION: Chinese patients with chronic lymphedema had moderate levels of QOL. The QOL and specific domains of patients were affected by different factors. Special attention and targeted interventions should be given to improve patients' QOL.

Asunto(s)

Linfedema , Calidad de Vida , Adaptación Psicológica , Estudios Transversales , Humanos , Linfedema/etiología , Apoyo Social , Encuestas y Cuestionarios

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Chronic lymphedema can lead to several long-term complications. The causes of lymphedema can be primary, due to a genetic source, or secondary to procedures, trauma, or other conditions. Primary hereditary lymphedema, as in the case of Milroy's disease, is rare. Because of the condition's rarity, case reports mostly involve presentations to monitor for. Here we document a case of Milroy's disease in a 70-year-old woman with recurrent left lung effusions.

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Lymphedema is a frequent debilitating condition among cancer patients. Daily supportive treatment may be necessary without long-term improvement. We describe two cases with chronic refractory lymphedema treated with fractional 10,600 nm CO2 laser. A 61-year-old female with locally advanced cervical cancer presented with postsurgical edematous swelling of the vulva and mons pubis and recurring cellulitis due to chronic lymphangiectasia. After six treatments of fractional CO2 laser, she noticed an 80% reduction of lymphorrea, swelling, and frequency of cellulitis. A 32-year old melanoma patient presented with refractory right lower leg lymphedema post right inguinal lymph node dissection and radiation. After fractional CO2 laser, she noted increased softness of her inguinal scar and a decrease of the lower leg edema. Fractional CO2 laser may be useful in addressing chronic refractory lymphedema. Further research should confirm our findings to consider fractional laser as a standard method in the treatment of chronic lymphedema.

Asunto(s)

Láseres de Gas , Linfedema , Melanoma , Adulto , Dióxido de Carbono , Celulitis (Flemón) , Edema , Femenino , Humanos , Láseres de Gas/uso terapéutico , Linfedema/etiología , Linfedema/cirugía , Persona de Mediana Edad

RESUMEN

Angiosarcoma (AS) is a rare and aggressive tumor with high rates of local recurrence and distant metastasis. Stewart-Treves syndrome (STS) is defined as AS arising in the setting of chronic lymphedema, and is extremely uncommon in the lower abdominal wall. Eribulin mesylate (eribulin) is a non-taxane microtubule inhibitor that has been approved in Japan for treating soft tissue sarcoma. The current study reports the case of a 76 year-old woman with STS in the lower abdominal wall who exhibited an excellent response to eribulin. Having undergone surgery and postoperative radiation therapy (RT) for cervical cancer 12 years earlier, the patient presented with a mass in her left lower abdominal wall, where chronic lymphedema had developed. Contrast-enhanced computed tomography revealed multiple enhancing nodules in the left lower abdominal wall and edema of the subcutaneous tissues in the whole lower abdomen. A histologic analysis of the specimens revealed AS, and she was diagnosed as STS. A total of 3 cycles of combination chemotherapy with gemcitabine and docetaxel were administered, but the patient discontinued treatment owing to severe adverse events. RT was performed for the tumor, but multiple reddish nodules appeared in the whole lower abdominal wall 3 months later. At this point, eribulin administration was offered. After 4 cycles of treatment, there was a clear reduction in the size of the nodules. All lesions were stable, no new lesions had developed, and the side effects of treatment were minor over the course of 1 year. The results reveal that eribulin may serve as a potential therapeutic option for the treatment of STS.

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Squamous cell carcinoma (SCC) arising with chronic lymphedema is a rare condition, though literature suggests a variety of malignant tumors associated with filariasis. We present a case of 70-year-old male patient with a history of penile and scrotal SCC of filarial origin. We here discuss the unusual association of carcinoma and filariasis and its surgical treatment.

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The main benefit of autologous therapies is its easier obtention and relatively lower concerns regarding ethical implications and patient safety. We conducted a systematic review of publications assessing the potential use of blood components (lymphocytes, red blood cells (RBCs), platelet-rich plasma (PRP)) as targeted therapy in the treatment of lymphedema. We hypothesized that blood components could be used as targeted therapy in the lymphedema treatment. We also conducted a comprehensive, systematic review of the published literature on the use of blood components as targeted therapies in the treatment of lymphedema using the PubMed database. Eligibility criteria excluded papers that aimed to investigate the correlation of inflammatory cells and the physio-pathogenesis of lymphedema. Abstracts, presentations, reviews, and meta-analyses were also excluded. From the 338 potential papers found in the literature, 11 studies fulfilled the eligibility criteria. Different types of targeted therapies were proposed, but the majority of papers investigated the potential use of lymphocytes (9/11). The use of PRP was investigated in two papers and the use of RBCs in one paper. Interestingly, six out of 11 studies were done on patients with lymphedema, but the most recent was published in 1999. The remaining publications were experimental studies on dogs, rats, or in vitro. The publications demonstrated positive outcomes for the delivery of lymphocytes and PRP in lymphedema treatment. Lymphocyte was the most common blood component investigated. Even though more than half of the papers found were conducted on patients, we noticed a scientific gap of more than 20 years on the topic.

RESUMEN

Lipoaspiration is a potential treatment for lymphedema; however, there is a lack of knowledge regarding the outcomes and benefits of this procedure in lower limb lymphedema. We aim to describe the outcomes of studies to date reporting the use of lipoaspiration in lower limb lymphedema. We searched the PubMed database for studies that evaluated the use of lipoaspiration for lower limb lymphedema. The keywords "lipoaspiration" AND "lymphedema," synonyms, and different combinations were used for the search. Only English studies were included. Eight studies met the inclusion criteria from a total of 129 articles. A volume reduction greater than 50% was found in all patients who underwent lipoaspiration for lower limb lymphedema. Complete volume reduction was found after four to five years of follow-up. A greater volume reduction was found for secondary lymphedema when compared to primary lymphedema. Finally, improvement was found in functionality, quality of life, and rate of infection. Lipoaspiration is recommended for patients with lower limb lymphedema in stages 2 and 3 of the disease, followed by controlled compressive therapy that maintains the volume reduction accomplished by the procedure.

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Although physiologic surgeries for lymphedema (i.e., lymphovenous bypass, vascularized lymph node transplantation) are becoming well established, unpredictable outcomes have still been reported in some studies. Therefore, authors have investigated ways to improve these surgery outcomes. The goal of our study was to conduct a comprehensive systematic review of targeted therapy administration in the surgical treatment of lymphedema. We conducted a comprehensive systematic review of the published literature on targeted therapies associated with lymphedema surgery using the PubMed database. Eligibility criteria excluded papers that reported surgical treatment of lymphedema without the use of targeted therapies and also papers describing targeted therapies in nonsurgical treatment of lymphedema. Abstracts, presentations, reviews, and meta-analyses were also excluded. Extracted data included the year of study, country, lymphedema model, surgical technique, targeted therapy agent, therapy delivery, findings, and outcomes. From 823 potential papers found in the literature, 10 studies fulfilled the eligibility criteria. All papers were experimental, and most of them on small animal model (7/10). Different targeted therapies were proposed, but all of them were associated with lymph node transplantation. The most common targeted therapy proposed mechanism was growth factor delivery (8/10). However, one paper used adipose-stem cell, and one paper proposed the use of sterile inflammation. The pooled publications assessing targeted therapy administration in the surgical treatment of lymphedema demonstrate encouraging data for positive outcomes. To date, all studies were experimental and related to lymph node transfer.

RESUMEN

The aim of the present study was to evaluate the magnetic resonance imaging (MRI) data of patients with Stewart-Treves Syndrome (STS), and to compare them with the corresponding pathological data. A total of 4,289 cases of secondary upper limb lymphedema (LE) from a single center, including 5 female cases of STS who underwent modified-radical post mastectomy, were included in the study. All cases were diagnosed pathologically, and by conventional and contrast-enhanced (ce)MRI scans. A total of 43 tumor nodules (Φ ≥4 mm) were examined. According to the immunohistochemical results, the 5 cases of STS were divided into 2 types; 4 patients exhibited STS type I [angiosarcoma (AS)] and 1 patient exhibited STS type 2 [mixed lymphangiosarcoma (mLAS)]. The MRI signal intensity (SI) of all the nodules was compared with the pathological results. In the T1WI sequences, all nodules showed isointensity compared with normal muscle tissues in the same image. However, in the T2WI sequences, there were the visually recognizable differences in the SI compared with the LE tissues in the two STS types. The SI of mLAS was more markedly decreased compared with that of AS. The SI of mLAS was closer to that of normal muscle tissues compared with the SI of AS. In the ceMRI, the SI of the tumor nodules was markedly increased in the two STS types, compared with non-enhancement imaging. Overall, MRI is a useful tool for the evaluation and diagnosis of STS. A chronic case of LE, in which MRI examination demonstrates nodules within the LE, may suggest a diagnosis of STS.

RESUMEN

Stewart-Treves syndrome (STS) is a rare cutaneous angiosarcoma that develops in chronic lymphedema. The majority of STS is described in the upper extremity after aggressive locoregional therapy for breast cancer and is rarely reported in lower extremities. A 68-year-old woman presented with a 3-month history of multiple purpuric tumorous plaques and nodules on the right posterior thigh. She had a history of radical hysterectomy with lymph node dissection and postoperative radiotherapy due to uterine cervical cancer 16 years ago. She received right total hip replacement surgery due to hip joint avascular necrosis 14 years ago. She had suffered from chronic leg edema, especially on the right side. Skin biopsy on the right posterior thigh showed irregular vascular channels lined by atypical endothelial cells. Special stains showed positivity for CD31, CD34, factor VIII, and D2~40, which are pan-vascular or lymphatic markers. She showed a pelvic mass and pelvic bone metastasis on radiologic staging work-up. She refused all treatment, including surgery, radiotherapy, and chemotherapy, except for pain control. She died 2 months after diagnosis of this highly malignant tumor. The lymphedema on both lower extremities after uterine cervical cancer treatment was aggravated especially on the right lower extremity after right total hip replacement surgery. Increased weight of the right lower extremity resulted in 4 episodes of recurrent hip dislocation. We contend that these multiple factors (uterine cervical cancer treatment, total hip replacement surgery on the right side, and recurrent hip dislocations) attributed to development of Stewart-Treves syndrome. We herein report a case of Stewart-Treves syndrome of the lower extremity following chronic leg lymphedema after uterine cervical cancer treatment and hip surgery.

Asunto(s)

Anciano , Femenino , Humanos , Artroplastia de Reemplazo de Cadera , Biopsia , Neoplasias de la Mama , Colorantes , Diagnóstico , Quimioterapia , Edema , Células Endoteliales , Factor VIII , Hemangiosarcoma , Cadera , Luxación de la Cadera , Articulación de la Cadera , Histerectomía , Pierna , Extremidad Inferior , Escisión del Ganglio Linfático , Linfedema , Necrosis , Metástasis de la Neoplasia , Huesos Pélvicos , Radioterapia , Piel , Muslo , Extremidad Superior , Neoplasias del Cuello Uterino

RESUMEN

INTRODUCTION: Use of indocyanine green (ICG) near-infrared fluorescence as a dye to assess tissue vascularization is now well standardized. The aim of this literature review was to review and resume the most recent recommendations for ICG use in its plastic surgery applications. METHODS: A systematic literature review was performed using Medline, EMBASE, and PubMed databases to obtain the latest recommendations for ICG in plastic surgery. Inclusion criteria were all articles written in English language that evaluated pre-, intra-, or postoperative ICG applications in surgical procedures usually performed by plastic surgeons. Case reports, reviews, meta-analyses, and experimental studies on animals or cadavers were excluded after title and abstract screening. RESULTS: Of the 1389 article titles retrieved, 41 full-text articles met the inclusion criteria. ICG applications in plastic surgery were ICG lymphangiography used in sentinel lymph node mapping for breast cancer and melanoma and in microsurgery for the staging and treatment of secondary chronic lymphedema. The latest updates of ICG angiography in assessing free flaps, pedicled flaps, or large skin paddles were also retrieved. CONCLUSIONS: Large prospective studies suggest that ICG lymphography could be used as a single tracer to reliably perform sentinel lymph node biopsy. In the case of cutaneous melanoma, ICG lymphography increases node detection sensitivity and accuracy in conjunction with lymphoscintigraphy. In chronic lymphedema, it is useful for pre- and postoperative staging and intraoperative anatomical location of lymphatic pathways when lymphovenous bypass is indicated. ICG angiography is used intraoperatively to assess free flap anastomosis and design skin paddles and postoperatively to monitor buried flaps. In pedicled perforator flaps or for large skin paddles, intraoperative ICG angiography is strongly correlated with postoperative outcomes. LEVEL OF EVIDENCE: 3.

Asunto(s)

Colorantes , Verde de Indocianina , Procedimientos de Cirugía Plástica/métodos , Neoplasias de la Mama/diagnóstico por imagen , Neoplasias de la Mama/patología , Neoplasias de la Mama/cirugía , Enfermedad Crónica , Femenino , Angiografía con Fluoresceína , Humanos , Linfedema/diagnóstico por imagen , Linfedema/cirugía , Linfografía/métodos , Melanoma/diagnóstico por imagen , Melanoma/patología , Melanoma/cirugía , Microcirugia/métodos , Biopsia del Ganglio Linfático Centinela/métodos , Neoplasias Cutáneas/diagnóstico por imagen , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/cirugía , Colgajos Quirúrgicos/irrigación sanguínea , Melanoma Cutáneo Maligno

RESUMEN

Retiform hemangioendothelioma (RH) is a rare vascular neoplasm with a high rate of local recurrence and low metastatic potential. We describe an unusual case of RH in a 45-year-old patient with Milroy disease, with a prominent solid component diffusely involving a chronic lymphedematous leg. This case is consistent with the postulated relationship between lymphedema and vascular neoplasms developing as a result of local immune dysfunction, and highlights the need to closely monitor patients with Milroy disease for pathologic changes. Our case highlights a unique example of RH with atypical features. There are several noteworthy unusual clinical and histologic findings including diffuse involvement of an entire limb, solid component with cytologic atypia, D2-40 expression, and first-time-reported association with Milroy disease. Given the atypical histologic presentation of cytologic atypia, solid areas and atypical immunohistochemical profile with D2-40 positivity, this case could cause diagnostic difficulty, especially in the setting of such a broad clinical differential.

Asunto(s)

Hemangioendotelioma/patología , Linfedema/complicaciones , Neoplasias Cutáneas/patología , Neoplasias Vasculares/patología , Femenino , Humanos , Persona de Mediana Edad

Asunto(s)

Hemangiosarcoma/diagnóstico , Linfangiosarcoma/diagnóstico , Linfedema/complicaciones , Adulto , Diagnóstico Diferencial , Resultado Fatal , Hemangiosarcoma/etiología , Hemangiosarcoma/terapia , Humanos , Extremidad Inferior/patología , Linfangiosarcoma/etiología , Linfangiosarcoma/terapia , Linfedema/diagnóstico , Masculino , Cuidados Paliativos/métodos , Pronóstico

RESUMEN

INTRODUCTION: Breast sarcomas are rare with an annual incidence of 4.6 cases/1,000,000 women. They can appear as primary forms or secondary to radiation therapy or chronic lymphedema. PRESENTATION OF CASE: A 41 year old woman attended our hospital after having noticed an increase in the size of her fibroadenoma. The examination revealed a 7cm retroareolar nodule. Breast sonography described a hypoechoic bilobulated lesion and MRI showed a large size polinodular image, suggesting a Phyllodes tumor. A core needle biopsy was performed with a histological result of low-grade fusiform cells sarcoma on Phyllodes tumor so we proceeded to surgical treatment with a mastectomy. After two years and a half she noticed a tough nodule over the mastectomy scar, which was resected with a histological result of fusiform cells sarcoma. Considering the diagnosis of recurrence of the disease, surgery was undertaken. DISCUSSION: Breast sarcoma is a rare but aggressive entity. Core biopsy is the procedure of choice for the diagnosis. Lymphatic spread is uncommon so nodal status in breast sarcoma is less informative. Staging study differs from other breast tumors and chest computed tomography is helpful since lungs are the predominant metastatic sites. The use of radiotherapy or chemotherapy is controversial and will depend on the risk of tumor recurrence. CONCLUSION: Surgery represents the only potentially curative therapy for breast sarcoma. Tumor size and adequate resection margin are the most important prognostic factors. Approximately 80% of recurrences appear in the first two years.

RESUMEN

El síndrome de Stewart-Treves es un angiosarcoma cutáneo, poco frecuente, que se produce en pacientes con linfedema crónico. Se describe clásicamente luego de realizar mastectomía radical con vaciamiento ganglionar y/o radioterapia. Su mortalidad es elevada. Comunicamos el caso de una paciente de 76 años de edad, sometida a mastectomía radical con vaciamiento ganglionar axilar, seguido de quimioterapia y radioterapia. Consulta 13 años después por dolor intenso y lesión tumoral exofitica, ulcerada, angiomatosa, sangrante y maloliente en cara anterior del brazo izquierdo. Presentaba edema del miembro de varios años de evolución. Luego de realizar biopsia por punch de la lesión, los resultados histopatológicos resultaron compatibles con linfangiosarcoma. La familia junto con el servicio de Cirugía decide realizar la extirpación del tumor, con conservación del miembro. Falleció luego de once meses de realizado el diagnóstico de síndrome de Stewart-Treves, en nuestro Servicio.

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The Stewart-Treves Syndrome is a rare cutaneous angiosarcoma, that occurs in patients with chronic lymphedema. It is classically described after performing radical mastectomy with lymph node dissection and/or radiation therapy. Its mortality is high. We have reported the case of a 76 years old patient, who underwent radical mastectomy with axillary lymph node dissection, followed by chemotherapy and radiotherapy. She came 13 years later with an intense pain and exophytic, ulcerated, angiomatous, bleeding and smelly tumor injury in front side of the left arm. She had an edema of the left upper limb with several years of evolution. After performing punch biopsy of the lesion the histopathological results were compatible with lymphangiosarcoma. The family, jointly with the surgery service decided to remove the tumor with limb preservation. She died 11 months after the diagnosis of Stewart-Treves syndrome, performed in our department.