Massive localised lymphoedema: a clinicopathological study of 22 cases and review of the literature

Manduch, Marosh; Oliveira, André M.; Nascimento, Antonio G.; Folpe, Andrew L.

doi:10.1136/jcp.2009.066555

Cited by 42 publications

(42 citation statements)

References 20 publications

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“…1,17 The gross findings were similar to the previous published data. 1,2,13 Sectioning of the masses demonstrated dramatic amount of edema and abundant mature adipose tissue separated by thick, edematous fibrous septae, a finding reported by others. 1,2,9 We also noted a unique pattern of dystrophic calcification in seven of our cases that somewhat mimicked hyperchromatic, atypical nuclei that may lead to a misdiagnosis of atypical lipomatous tumor/well-differentiated liposarcoma.…”

Section: Discussionsupporting

confidence: 55%

“…[28][29][30] In summary, we report that massive localized lymphedema is a benign, reactive, superficial pseudoneoplastic lesion seen mostly in morbidly obese patients and most commonly involving the thigh, similar to others. 1,2 Unique to this study is the tendency for multiplicity, a novel case of involvement of perianal region, and a peculiar pattern of dystrophic calcifications that can lead to a misdiagnosis of atypical lipomatous lesions/well-differentiated liposarcomas. In these cases, a von Kossa special stain can be reassuring.…”

Section: Discussionmentioning

confidence: 99%

“…The other differential diagnoses can include desmoid-type fibromatosis, lipomatosis, angiomatosis, lymphangiomatosis, angiosarcoma, angiomyoma, lipoma, and lipomatosis. 1,2,8 However, careful attention to the clinical history and prototypic histomorphology will facilitate prompt recognition and avoid major mishaps.…”

Section: Discussionmentioning

confidence: 99%

“…Others have noted a similar propensity for misdiagnoses as atypical lipomatous tumor/well-differentiated liposarcoma. 1,2 Helpful diagnostic clues include the history of morbid obesity and prominent skin changes characteristic of massive localized lymphedema, findings not described in atypical lipomatous tumor/ well-differentiated liposarcoma. The other differential diagnoses can include desmoid-type fibromatosis, lipomatosis, angiomatosis, lymphangiomatosis, angiosarcoma, angiomyoma, lipoma, and lipomatosis.…”

Section: Discussionmentioning

confidence: 99%

“…Massive localized lymphedema displays a predilection for the inner thigh, but other sites have been reported, including the lower abdominal wall, suprapubic region, mons pubis, vulva, inguinoscrotal, penis, popliteal fossa, lower leg, and arm. [1][2][3][4][5][6][7][8][9] The etiology and pathogenesis of massive localized lymphedema is unclear but might be multifactorial. In the original paper of Farshid and Weiss, lymphatic obstruction due to massive adipose tissue was proposed as the main etiological factor.…”

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confidence: 99%

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Massive localized lymphedema: a clinicopathologic study of 46 patients with an enrichment for multiplicity

et al. 2016

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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.

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Section: Discussionsupporting

confidence: 55%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Massive localized lymphedema: a clinicopathologic study of 46 patients with an enrichment for multiplicity

et al. 2016

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Disorders of the Lymphatic Vessels

Mortimer

Gordon

2016

Rook's Textbook of Dermatology, Ninth Edition

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Lymphatic dysfunction interferes with fluid homeostasis, tissue immunity and peripheral fat mobilization. Any chronic oedema represents lymphatic failure. If impaired lymph drainage is solely responsible, then lymphoedema results. This produces characteristic skin changes known as elephantiasis as well as increased fat deposition in the subcutaneous tissues. Impaired immune cell trafficking results in an increased risk of infection, particularly cellulitis/erysipelas, which often becomes recurrent. This chapter describes the clinical consequences of lymphatic dysfunction and in particular the impact on the skin and subcutaneous tissues. The sections are divided according to common clinical presentations.

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Disorders of the Lymphatic System

Mortimer,

Gordon

2024

Rook's Textbook of Dermatology

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The lymphatic system is the body’s cleansing and recycling service. It removes toxins, waste and recycles fat and fluid. It also forms part of our immune system. The lymphatic system provides all the checks and balances for other body systems. It is the main controller of body fluid balance and it provides immunosurveillance and is responsible for creating immunity. It mobilises fat: impaired lymph drainage results in fat deposition and lymphoedema swelling is as much fat as fluid. It is also important for preventing atherosclerosis. It patrols for cancer cells; if this surveillance fails, cancer progresses. It regulates self‐tolerance, with failure resulting in autoimmune disease.

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Massive localised lymphoedema: a clinicopathological study of 22 cases and review of the literature

Abstract: The diagnosis of MLL continues to be challenging, in particular for pathologists. Awareness of this entity, clinical correlation and gross pathological correlation are essential in the separation of this distinctive pseudosarcoma from its various morphological mimics.

Cited by 42 publications

References 20 publications

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

Disorders of the Lymphatic Vessels

Disorders of the Lymphatic System

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