Massive Localized Lymphedema: A Clinicopathologic Study of 35 Patients with an Enrichment for Multiplicity
Habibe Kurt, Christina A Arnold, Obiajulu H Iwenofu. Ohio State University, Columbus, OH
Background: Massive localized lymphedema (MLL) is a monstrous tumefactive pseudosarcoma seen in middle aged morbidly obese adults. MLL displays a predilection for the inner thigh but other sites have been reported. Despite its initial description in 1998, the etiology remains unknown although associations with trauma, surgeries, and hypothyroidism have been reported. Herein, we report the largest study of MLL and expand upon its clinicopathologic features.
Design: Electronic medical records uncovered 35 patients with “massive localized lymphedema' from the institutional archives of The Ohio State University Wexner Medical Center between 2002 – 2012. All available H&E stained slides were reviewed.
Results: 35 patients (18 males and 17 females, age range 23-76 years, mean 49 years) presented with large soft tissue masses developing over a 5-36 months period. The majority of patients were Caucasian (n=30) with a Caucasian:African American ratio of 6:1. All patients were obese with a mean weight of 390.61 lb (range 209– 576 lb) and a mean BMI of 60.42 (range 36-81.35 kg/m2). Endocrinopathies were present in the majority (n=18): diabetes mellitus type II (n=11), hypothyroidism (n=5), and both diseases (n=2). 2 patients had an antecedent history of trauma and 8 presented with multifocal MLL. The tissue sites included thigh (n=24), abdomen (n=12), mons pubis (n=5), scrotum (n=2), perianal (n=1) and right flank (n=1). Mostly, the clinical impression was lymphedema pseudotumor. Only 2 patients underwent MRI to rule out sarcoma. Grossly, the mean weight was 6401 grams (range 740-26940gr) and the mean size was 54 cm (range 14.8-147.6cm). Histologically, all cases showed prototypic features of MLL including variable degrees of cutaneous reactive changes, alternating edema and fibrosis of the dermis punctuated by foci of lymphangiectasia, and expansion of the fibrous septae encasing lobules of fat in the subcutis with variably atypical spindle cells.
Conclusions: We report the largest series of MLL. As MLL remains an important mimic for atypical lipomatous lesions/well-differentiated liposarcomas, it is crucial to be aware of its peculiar clinicopathologic features. Our data demonstrate this is a lesion exclusively seen in morbidly obese patients where the thigh is the most common site of involvement. We note for the first time, a marked racial predilection for Caucasians and a tendency towards multiplicity. Approximately 50% of patients in our series have an associated endocrinopathy, suggesting that these may be part of the metabolic syndrome of obesity and could play a role in its pathogenesis.
Category: Bone & Soft Tissue
Monday, March 4, 2013 1:00 PM
Poster Session II # 22, Monday Afternoon