Massive Localized Lymphedema With Unusual Presentations: Report of 2 Cases and Review of the Literature

Department of Pathology, The University of Texas Southwestern Medical Center, Dallas, TX 75390-9072, USA.
International Journal of Surgical Pathology (Impact Factor: 0.96). 08/2008; 19(2):212-6. DOI: 10.1177/1066896908320833
Source: PubMed

ABSTRACT Massive localized lymphedema is a benign soft tissue lesion that usually presents as a large mass in morbidly obese adults. The diagnosis may be challenging as it can mimic other lesions, including well-differentiated liposarcoma. We report 2 cases of massive localized lymphedema with unusual presentation. The first case is a recurrent massive localized lymphedema in the right thigh of a 40-year-old morbidly obese woman. In addition to typical massive localized lymphedema features such as prominent edema and vascular proliferation in the adipose tissue, we observed prominent and abundant multinucleated stromal floret-like giant cells, arborizing network of capillaries, and areas of hyalinized collagen. Our second case is in a rare location (scrotum extending into penile soft tissue) in an overweight 55-year-old male. This lesion exhibits striking smooth muscle hyperplasia. Lack of staining by antibodies against murine double minute 2 protein and cyclin dependent kinase 4 and absence of high mobility group AT- hook 2 transcription factor rearrangement by fluorescence in situ hybridization support our diagnosis of massive localized lymphedema in both cases.

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    ABSTRACT: 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 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 was extracted. Prior infection of the scrotal lymphedema, surgical details, pathologic evaluation, and post-operative complications were noted. A series of the cases is presented. Results Four cases met the criteria for study. Average age was 35 years with an average body mass index of 53.9. Average resection at the first procedure was 3492 grams. All patients were reconstructed with laterally based scrotal flaps. 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 massive localized lymphedema 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.
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    ABSTRACT: Massive localized lymphedema (MLL) is a recently described entity associated with obesity, most often on the thighs. It rarely occurs on the vulva, and it may pose diagnostic difficulty for both clinician and pathologist in this setting. An obese 55-year-old woman underwent excision of bilateral polypoid vulvar masses. Histology confirmed the diagnosis of MLL. Although MLL is not common on the vulva, with the increasing incidence of obesity in the population, more cases can be anticipated, and an awareness of this lesion is important.
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    ABSTRACT: 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. We sought to evaluate the clinical and histopathologic features. The clinicopathologic features in patients with localized lymphedema of the genital region were studied. 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). Only 18 cases were included in our study. 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.
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