Massive localized lymphedema: a clinicopathologic study of 46 patients with an enrichment for multiplicity
Massive localized lymphedema: a clinicopathologic study of 46 patients with an enrichment for multiplicity
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DOI：
10.1038/modpathol.2015.135
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发表时间：
2016-01-01
期刊：
影响因子：
7.5
通讯作者：
Iwenofu, O. Hans
中科院分区：
文献类型：
--
作者：
Kurt, Habibe;Arnold, Christina A.;Iwenofu, O. Hans
大规模局部淋巴水肿是一种巨大的肿胀性假肉瘤，见于中年病态肥胖成年人。自 1998 年首次描述以来，尽管有报道称其与外伤、手术和甲状腺功能减退症有关，但病因仍不清楚。在此，我们报告了大规模局部淋巴水肿的最大研究，并扩展了其临床病理特征。从 2002 年至 2015 年间俄亥俄州立大学韦克斯纳医学中心的机构档案中回顾性地发现了 46 名患者的 54 例病例。46 名患者（21 名男性和 25 名女性，平均年龄 50 岁）在 5 至 60 个月的时间内出现了大块肿块。大多数患者是白种人（n = 39）。所有患者均肥胖，平均体重为 384.7 磅，平均体重指数为 59.6 kg/m(2)。 36 名患者有动脉粥样硬化性心血管疾病史，22 名患者患有 2 型糖尿病。八名患者患有多灶性大规模局部淋巴水肿。这些部位包括大腿 (n = 33)、腹部 (n = 17)、耻骨上区域 (n = 1)、阴阜 (n = 6)、阴囊 (n = 2)、肛周区域 (n = 1) 和右侧胁腹 (n = 1)。大多数情况下，临床印象是良性过程，包括血管翳或淋巴水肿假瘤。总体而言，平均重量为 8237 克，平均尺寸为 53.2 厘米。组织学上，八个病例显示出一种独特的营养不良性钙化模式，类似于深染、非典型细胞核，可能导致脂肪肉瘤的误诊，四例显示局灶性化生骨化，三例除了大规模局部淋巴水肿的原型特征外还显示多核细胞。我们报告这是最大的大规模局部淋巴水肿系列。这种病变最常见于病态肥胖患者，大腿是最常见的受累部位。我们注意到对白种人的明显种族偏好和多元化倾向。我们认为肥胖本身和相关的代谢综合征在其发病机制中发挥着重要作用。
Massive localized lymphedema is a monstrous tumefactive pseudosarcoma seen in middle-aged morbidly obese adults. Since 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 massive localized lymphedema and expand upon its clinicopathologic features. Fifty-four cases from 46 patients were retrospectively identified from the institutional archives of The Ohio State University Wexner Medical Center between 2002 and 2015. Forty-six patients (21 males and 25 females, mean age 50 years) presented with large masses developing over a 5-60-month period. The majority of patients were Caucasian (n = 39). All patients were obese with a mean weight of 384.7 lb and a mean body mass index of 59.6 kg/m(2). Thirty-six patients had a history of atherosclerotic cardiovascular disease and diabetes mellitus type 2 was present in 22 patients. Eight patients had multifocal massive localized lymphedema. The sites included thigh (n = 33), abdomen (n = 17), suprapubic region (n = 1), mons pubis (n = 6), scrotum (n = 2), perianal region (n = 1), and right flank (n = 1). Mostly, the clinical impression was benign processes, including pannus or lymphedema pseudotumor. Grossly, the mean weight was 8237 g and the mean size was 53.2 cm. Histologically, eight cases showed a unique pattern of dystrophic calcifications mimicking hyperchromatic, atypical nuclei that might lead to misdiagnosis of liposarcoma, four cases showed focal metaplastic ossification, and three cases showed multinucleated cells in addition to prototypic features of massive localized lymphedema. We report that this is the largest series of massive localized lymphedema. This is a lesion mostly seen in morbidly obese patients and the thigh is the most common site of involvement. We note a marked racial predilection for Caucasians and a tendency towards multiplicity. We suggest that obesity itself and the related metabolic syndrome have an important role in its pathogenesis.