Acta Dermato-Venereologica 99-4CompleteContent | Page 24

452 SHORT COMMUNICATION Massive Localized Lymphedema, an Emerging Problem and a Challenging Pseudosarcomatous Condition Mimicking Liposarcoma Aleksandra G. FLOREK 1 , Rami LUTFI 2 , Kenneth WIND 3 , Wenhua LIU 4 and Aleksandar L. KRUNIC 5 Department of Dermatology, University of Colorado Denver School of Medicine, 1665 Aurora Court, Denver CO 80045, 2 Department of General Surgery and 4 Department of Dermatology, University of Illinois, Chicago, Chicago, IL, and 3 Department of Pathology, Presence Saint Joseph Hospital, Chicago, IL, USA. E-mail: [email protected] 1 Accepted Dec 6, 2018; E-published Dec 6, 2018 Massive localized lymphedema (MLL) is an emerging complication of the obesity epidemic and a challenging clinical entity mimicking liposarcoma. It presents as an ill-defined mass of the proximal medial extremities in morbidly obese adults (1). MLL is caused by the compression of regional lymphatic vessels by massive adipose tissue (1–3). Although benign, the diagnosis can be challenging since atypical lipomatous tumors and liposarcomas present in the same manner (2, 3). Furthermore, MLL can transform to an angiosarcoma, in a phenomenon known as Stewart-Treves syndrome (2, 3). This case highlights the need for clinical vigilance for dermatologists to recognize this entity morpholo- gically and to institute appropriate investigation and management. history, presented with a one-year duration of a gradually enlar- ging, asymptomatic swelling of the left upper inner thigh. Phy- sical exam revealed an ill-defined, skin-colored, firm mass with a woody induration, peau d’orange appearance, and non-pitting edema (Fig. 1a). An incisional biopsy revealed a markedly edematous dermis with increased ectatic lymphatic channels, spindle-shaped cells, floret-like multinucleated cells, and atypical stromal spindle cells (Fig. 2a). The spindle-shaped cells and floret-like multinucleated cells were positive for CD34 (Fig. 2b) and negative for CD31 and smooth muscle actin. These findings were most consistent with massive localized lymphedema (MLL). The patient underwent wide excision of the tumor (21.8 × 14.9 × 4.6 cm; 831.1 g) with clear margins confirmed on histopathology (Fig. 1b). Over the next 12 months, patient was monitored closely and showed no recurrence of the tumor. CASE REPORT A 52-year-old morbidly obese woman (weight 145.9 kg, body mass index 53.4 kg/m 2 ) with no significant medical or surgical Massive localized lymphedema is a rare entity that presents with long-standing, painless, slow-growing, ill-defined soft tissue mass in middle age morbidly obese Fig. 1. a) Ill-defined, non–tender, skin-colored, pendulous mass on the left upper inner thigh with thick, firm, woody induration, peau d’orange appearance, and non-pitting edema. b) The patient underwent wide excision of the tumor (21.8 × 14.9 × 4.6 cm; 83.1 g) with clear margins confirmed on histopathology with identical findings to original incisional biopsy. DISCUSSION Fig. 2. a) Markedly edematous dermis with increased ectatic lymphatic channels. There were increased spindle-shaped cells and floret-like multinucleated cells, as well as atypical stromal spindle cells (hematoxylin and eosin, ×  400). b) The spindle-shaped cells and floret-like multinucleated cells were positive for CD34 stain (CD34 stain, magnification × 400). doi: 10.2340/00015555-3106 Acta Derm Venereol 2019; 99: 452–453 This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica.