Case 143: Madelung Disease

Landis, Mark; Etemad-Rezai, Roya; Shetty, Karan; Goldszmidt, Mark

doi:10.1148/radiol.2503071024

Cited by 17 publications

(10 citation statements)

References 8 publications

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“…This disease has typical clinical and imaging appearances and is not difficult to diagnose. The distribution of the fatty deposits distinguishes Madelung disease from obesity (6,34).…”

Section: Lipoblastomamentioning

confidence: 99%

Soft Tissue Tumors of the Head and Neck: Imaging-based Review of the WHO Classification

Razek

Huang²

2011

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126

108

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The World Health Organization (WHO) system for defining and classifying soft tissue tumors is usually applied to lesions that occur in the trunk and extremities, but it also provides an excellent framework for characterizing nonepithelial extraskeletal tumors of the head and neck. Although nonepithelial extraskeletal tumors are in the minority among head and neck lesions, they are by no means rare. The WHO classification system recognizes nine major types based on histologic differentiation: adipocytic, fibroblastic or myofibroblastic, fibrohistiocytic, smooth muscle, skeletal muscle, vascular, pericytic, and chondro-osseous tumors, as well as soft tissue tumors of uncertain differentiation. Tumors of each histologic type may be further subclassified on the basis of their biologic behavior as benign, intermediate (ie, having malignant potential), or malignant. Imaging plays an important role in the noninvasive diagnosis and characterization of nonepithelial soft tissue tumors of the head and neck, providing clues about tumor grade, composition, extent, and involvement of adjacent structures. Although the imaging characteristics of many such tumors are nonspecific, consideration of the clinical history in concert with the imaging findings may help limit the differential diagnosis or even allow reliable diagnosis of some of these tumors.

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“…This disease has typical clinical and imaging appearances and is not difficult to diagnose. The distribution of the fatty deposits distinguishes Madelung disease from obesity (6,34).…”

Section: Lipoblastomamentioning

confidence: 99%

Soft Tissue Tumors of the Head and Neck: Imaging-based Review of the WHO Classification

Razek

Huang²

2011

RadioGraphics

126

108

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“…Madelung's disease is a rare disorder which predominantly aff ects males between the ages of 30 and 60 years [8]. It is characterized by the presence of massive multiple symmetrical non-encapsulated fat accumulations [9].…”

Section: Discussionmentioning

confidence: 99%

Osteoporosis, weight gain and atypical fat accumulations – a typical feature not only for Cushing's, but also Madelung's disease: A case report

Friedl

Sampl

Prandl

et al. 2011

Wien Klin Wochenschr

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Madelung's disease, or multiple symmetric lipomatosis, is an extremely rare disease and very likely to be under-diagnosed. It is characterized by multiple symmetrical non-encapsulated fat accumulations mainly located around the neck, shoulders, upper extremities and upper parts of the trunk. The disorder predominantly affects middle-aged men of Mediterranean origin with a history of ethanol intake. Reports of this uncommon disorder in women are very rare. The aim of this article is to report the endocrine and metabolic workup leading to the diagnosis of this uncommon disorder. A 55-year-old woman with osteoporosis was referred to our outpatient clinic with a suspected diagnosis of Cushing's disease. The patient complained of undesired weight gain with atypical fat accumulations predominantly in the upper trunk region during the previous 10 weeks. She presented with the characteristic physical features of Madelung's disease and underwent a thorough examination with endocrine and metabolic evaluation of this rare condition and was finally diagnosed with Madelung's disease. This report demonstrates how a diagnosis of this rare disorder can be reached efficiently. A history of osteoporosis in combination with weight gain and atypical fat accumulations ultimately led to the diagnosis of Madelung's diseases, a rather unknown disorder likely to be under-diagnosed. Although treatment options are limited, a diagnosis is still important for the affected individual.

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“…Alcohol cessation and weight control are recommended although they do not reverse or stop the course of the disease [35]. MSL is also found in non-alcoholics in association with mitochondrial DNA mutations [36]. A defect of adrenergic stimulated lipolysis or mitochondrial disorder of brown fat tissue has been considered as the etiology of this disease in recent studies [37].…”

Section: Insulin Injectionmentioning

confidence: 99%

Imaging spectrum of abnormal subcutaneous and visceral fat distribution

et al. 2020

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Adipose tissue plays multiple and complex roles not only in mechanical cushioning and energy storage but also as an important secretory organ that regulates energy balance and homeostasis multilaterally. Fat tissue is categorized into subcutaneous fat tissue (SCAT) or visceral fat tissue (VSA) depending on its distribution, with the two having different metabolic functions. Near-total lack of fat in congenital/acquired generalized lipodystrophy, cachexia, or any other severe malnutrition condition induces severe multi-organ dysfunction due to lack of production of leptin and other adipokines. Increased visceral fat tissue secondary to obesity, hypercortisolism, or multiple symmetric lipomatosis raises the risk of insulin resistance, cardiac complications, and airway or spinal canal stenosis, although the fat distribution pattern differs in each condition. Partial abnormal fat distribution conditions such as HIV/HAART therapy-associated lipodystrophy, familial partial lipodystrophies, and acquired partial lipodystrophy frequently show a mixture of lipoatrophy and lipohypertrophy with metabolic dysfunction. Characteristic imaging features in conditions with local abnormal fat distribution can provide information about a patient's co-existent/unrecognized disease(s), past medical history, or lifestyle. Knowledge of characteristic abnormal fat distribution patterns can contribute to proper and timely therapeutic decision-making and patient education.

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Case 143: Madelung Disease

Cited by 17 publications

References 8 publications

Soft Tissue Tumors of the Head and Neck: Imaging-based Review of the WHO Classification

Soft Tissue Tumors of the Head and Neck: Imaging-based Review of the WHO Classification

Osteoporosis, weight gain and atypical fat accumulations – a typical feature not only for Cushing's, but also Madelung's disease: A case report

Imaging spectrum of abnormal subcutaneous and visceral fat distribution

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