Massive localized lymphedema (MLL) is a rare disease caused by the obstruction of lymphatic vessels with specific clinical morphological and radiological characteristics. People with morbid obesity are mainly affected by MLL. Lymphedema is easily confused with soft tissue sarcoma and requires differential diagnosis, both the possibility of an MLL and also carcinoma manifestations in the soft tissues. The possible causes of massive lymphedema include trauma, surgery, and hypothyroidism. This report is the first case of MLL treated surgically in the Russian Federation. Detailed computed tomography (CT) characteristics and an electron microscope picture of MLL are discussed. A 50-year-old woman (body mass index of 43 kg/m 2 ) with MLL arising from the anterior abdominal wall was admitted to the hospital for surgical treatment. Its mass was 22.16 kg. A morphological study of the resected mass confirmed the diagnosis of MLL. We review etiology, clinical presentation, diagnosis, and treatment of MLL. We also performed an electron-microscopic study that revealed interstitial Cajal-like cells telocytes not previously described in MLL cases. We did not find similar findings in the literature. It is possible that the conduction of an ultrastructural examination of MLL tissue samples will further contribute to the understanding of MLL pathogenesis.

Massive localized lymphedema (MLL) is an emerging clinical phenomenon predominantly observed in morbidly obese individuals. It presents both diagnostic and therapeutic challenges to clinicians due to its characterization by large, pendulous masses in the abdomen or thigh. MLL may resemble malignant conditions, such as liposarcoma, leading to unnecessary invasive interventions. This study presents two case studies: a 74-year-old male who succumbed to postoperative complications and a 56-year-old female who experienced successful recovery. These cases highlight the urgent need for robust diagnostic criteria and evidence-based management approaches for MLL. In addition, further research exploring the pathogenesis, risk factors, and potential connections among MLL, hypothyroidism, and angiosarcoma is essential.

Massive localized lymphedema (MLL) is a rare disease observed in people with morbid obesity. MLL is easily confused with soft tissue sarcoma. Therefore, MLL is sometimes called as pseudosarcoma in the literature. MLL was initially described by G. Farshid and S. Weiss in 1998. However, etiology of MLL is still unknown despite certain relationships with injuries, operations and hypothyroidism. Since the term «MLL» was introduced only 20 years ago, there are no reliable statistical data on the prevalence of MLL. According to the World Health Organization data (2016), 13% of the adult world population are obese. Therefore, the risk of MLL is increased in these people. Thigh is the most common site of lesion. Abdominal wall lesion is rare and can cause diagnostic difficulties due to large dimensions and appearance. We report a 50-year-old obese woman with MLL of anterior abdominal wall. She experienced discomfort while walking and sleeping due to giant MLL of anterior abdominal wall that significantly reduced quality of life. Removal of MLL (weight 22160 g) was followed by favorable cosmetic and functional outcome. Analysis of differences between MLL and soft tissue sarcoma in the era of «obesity epidemic» is valuable for correct diagnosis and treatment of this rare complication. This report is the first case of MLL, registered in Russia. In our opinion, this is associated with insufficient awareness of physicians about this complication of obesity. We hope our observation will help clinicians to identify and treat this complication.
Массивная локализованная лимфедема (МЛЛ) — редкое заболевание, наблюдается у людей с патологическим ожирением, ее легко спутать с саркомой мягких тканей, в связи с этим в литературе она упоминается под названием «псевдосаркома». С первоначального описания G. Farshid и S.W. Weiss в 1998 г. этиология МЛЛ остается неизвестной, хотя сообщается о связях с травмой, операциями и гипотиреозом. Поскольку термин «массивная локализованная лимфедема» ввели лишь 20 лет назад, достоверных статистических данных о распространенности МЛЛ нет. По данным ВОЗ 2016 г., 13% взрослого населения Земли страдают ожирением, соответственно растет и риск развития МЛЛ у этой группы людей. Бедро — наиболее частый участок поражения. Локализация на брюшной стенке встречается редко и может вызвать диагностические трудности из-за больших размеров и внешнего вида. Приводим клиническое наблюдение МЛЛ брюшной стенки у женщины, 50 лет, с ожирением. Из-за гигантских размеров образования передней брюшной стенки она испытывала дискомфорт во время ходьбы и сна, что значительно снижало качество жизни. После удаления этого образования массой 22 160 г достигнут хороший косметический и функциональный результат. Описание различий между МЛЛ и саркомой мягких тканей в эпоху «эпидемии ожирения» позволяет правильно диагностировать и лечить это редкое осложнение. Данное сообщение является первым случаем МЛЛ, зарегистрированным в России, что, на наш взгляд, связано с недостаточной осведомленностью врачей об этом осложнении ожирения. Надеемся, что наше наблюдение поможет клиницистам выявлять и лечить это осложнение.

We report a case of a 70-year-old woman with a BMI of 58 who developed cellulitis refractory to treatment, within an area of massive localized lymphedema. Biopsy showed angiosarcoma. MRI showed multiple lobulated, low T1, high T2 masses within a background of prominent soft tissue septal stranding, dilated lymphatic channels, and skin thickening. CT also showed the mass well, within the background lymphedema. Massive localized lymphedema is increasing in prevalence due to the worsening obesity epidemic. Radiologists should be aware that the presence of a nodule within an area of massive localized lymphedema is suspicious for sarcoma.

BACKGROUND: Massive localized lymphedema (MLL) is a non-neoplastic benign soft tissue lesion that may be confused with sarcomas or other neoplastic proliferations both clinically and morphologically. Most occur in morbidly obese adults on the lower extremities. The objective of this article is to document a case of MLL in the retroperitoneal cavity which is a previously unreported site for this lesion, and to highlight its unusual clinical features.
METHODS: The patient was a non-obese male who had undergone major abdominal surgery due to bladder extrophy 17 years ago. Abdominal ultrasonography detected a large incidental mass in the right renal sinus during his investigation for nephrolithiasis. The lesion extending from renal pelvis down to pelvis was resected and its histopathological findings were compatible with massive localized lymphedema.
CONCLUSIONS: Retroperitoneum has to be added to the list of locations that MLL can be found. Liposarcoma will be a challenging differential diagnosis when the lesion is encountered in an unusual site.

Massive localized lymphedema represents a pseudosarcoma seen most commonly in middle-aged morbidly obese patients that radiologically can be easily confused for a soft-tissue sarcoma if one is not familiar with this entity. Although considered relatively rare, as the obesity epidemic continues to rise, the incidence of this entity will likely increase as well. We present a case of massive localized lymphedema occurring in the medial thigh, the most common location, with imaging and pathologic correlation.

Massive localized lymphedema, also called pseudosarcoma, is a rare condition associated with morbid obesity. Accurate identification of this entity helps the physician make the distinction between this condition and other soft tissue tumors, especially with the increasing rate of obesity worldwide. Obesity and increased caloric intake lead to storage of the excess energy in the form of adipose tissue. The excess adipose tissue disturbs the lymphatic vessels, leading to massive edema localized mostly in the lower extremity, reaching a substantial size and weight that interferes with the quality of life of the individual in question. The mass can cause cosmetic but more importantly functional and structural defects, leading to altered biomechanics with increased risk of deep vein thromboembolism. Below is a presentation of a patient presenting with complaint of a massive medial thigh mass. After much investigation, consultation, and use of diagnostic radiologic modalities it was diagnosed as what is referred to in the literature as massive localized lymphedema, or pseudosarcoma. This mass was managed by surgical excision and the diagnosis was further confirmed by pathologic analysis. After the excision, our patient regained his ability to ambulate on his own, with the help of a rehabilitation program and physical therapy. Massive localized lymphedema needs to be on the differential diagnosis of any morbidly obese patient presenting with mass, especially in the lower extremities. Adequate knowledge about this condition enables the physician to make the distinction between pseudosarcoma and true soft tissue sarcomas.

BACKGROUND: Massive localized lymphedema (MLL) is a rare entity first described in 1998 in patients with morbid obesity; the incidence is rising with the increased prevalence of morbid obesity. This report defines the clinical presentation and surgical challenges in 6 patients with MLL.
METHODS: The MLL in 6 patients with morbid obesity (weight range 270 to 585 lbs) involved the thigh in 3 patients, the calf in 1 patient, and the abdomen in 2 patients. The time from onset to presentation averaged 3 years (range 1 to 8 years). Two thigh lesions precluded ambulation because both legs could not be on the ground simultaneously; the 2 abdominal lesions were too heavy to permit ambulation.
RESULTS: The surgical excision required the use of pulleys to elevate the MLL tissues, which, on excision, weighed between 24 and 78 lbs. A long oval horizontal incision and a long transverse incision were used for the 2 abdominal lesions. Long horizontal oval limb incisions with multiple perpendicular cross incisions had to be used to excise MLL in the 4 limb lesions. In 2 cases, the vessel-sealing device was employed successfully for dissecting subcutaneous edematous tissue. Loose wound closure permitted postoperative lymph leakage, which continued for 3 to 8 weeks. The histology demonstrated fibrotic lymphatic tissue with vascular and lymphatic proliferation and edema; all patients did well.
CONCLUSIONS: MLL is rare and is best treated by surgical excision facilitated by pulleys and imaginative incisions to obtain primary closure. Long-term follow-up is necessary to assess for subsequent liposarcoma or angiosarcoma.

BACKGROUND: Lymphedema of the penis and scrotum is physically and psychologically disabling. Obesity is a source of secondary lymphedema. When restricted to specific anatomical regions in obesity, this is termed massive localized lymphedema (MLL). Few surgical cases of specific scrotal MLL in obesity are reported in the literature. We present our case series to improve the management of this complicated pathology.
METHODS: This is a retrospective review of obese adult patients with clinically diagnosed scrotal MLL undergoing reduction scrotoplasty by the senior author (J.R.S.) from 1992 to 2012. Medical, social, familial, surgical, and follow-up data were extracted. Prior infection of the scrotal lymphedema, surgical details, pathologic evaluation, and postoperative complications were noted. A series of the cases is presented.
RESULTS: Four cases met the criteria for study. The average age was 35 years with an average body mass index of 53.9. Average resection at the first procedure was 3492 g. All patients were reconstructed with laterally based scrotal flaps. The pathology for each case was consistent with chronic lymphedema; no sarcomatous changes were noted. Fifty percent of the patients had recurrence of the scrotal MLL. The average total number of operations during the follow-up period for either complication or recurrence was two.
CONCLUSIONS: This is the largest case series specifically investigating surgical treatment for scrotal MLL in obesity. Lateral-based scrotal flaps (with or without mid-raphe Z-plasty) permit anatomic reconstruction. Complications are common and recurrence is frequent after surgical management. Excision with reconstruction improves urinary function and overall symptoms.

BACKGROUND: Localized lymphedema is a nonneoplastic condition associated with obesity and predominantly involving the legs. This condition has distinctive clinical and histologic features and only rarely has been mentioned in the dermatologic literature.
OBJECTIVE: We sought to evaluate the clinical and histopathologic features.
METHODS: The clinicopathologic features in patients with localized lymphedema of the genital region were studied.
RESULTS: We identified 18 patients with localized lymphedema clinically presenting as large polypoid or verrucous lesions. The patients were 5 men and 13 women with a mean age of 46.5 years. Twelve patients were obese at diagnosis. Thirteen patients presented with tumors involving the vulva, 4 patients with tumors in the penis and scrotum, and 1 patient with scrotal and pubic lesions. Histologically, all cases showed marked dermal edema along with dilated lymphatic spaces, fibroplasia, and verrucous epidermal changes (papillomatous and hyperplastic epidermis).
CONCLUSIONS: Only 18 cases were included in our study.
CONCLUSIONS: This condition is an uncommon and recently described entity that could potentially be clinically and histologically misdiagnosed as a neoplasm; thus, it needs to be included in the differential diagnosis of polypoid and verrucous skin tumors with extensive dermal edema and fibroplasia.