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Soft Tissue Tumors

Adipose tissue

Lipomatosis


Reviewer: Charanjeet Singh, M.D. (see Reviewers page)
Revised: 21 December 2012, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

See also pelvic lipomatosis

General
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● Diffuse overgrowth of mature adipose tissue

Epidemiology
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● Rare disorder of children under 2 years or adults
● Associated with obesity, Cushing’s disease, steroid therapy, protease inhibitors for HIV
● Familial multiple lipomatosis: rare, usually autosomal dominant; multiple lipomas of trunk and extremities with relative sparing of the head and shoulders; due to translocation involving high-mobility-group protein isoform I-C on chromosome 12 and the lipoma preferred partner gene on chromosome 3 (An Bras Dermatol 2012;87:324)
● See also below - adiposis dolorosa, multiple symmetric lipomatosis

Sites
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● Limb (may cause massive enlargement) or trunk

Case reports
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● Infiltrating lipomatosis in face of child (J Plast Reconstr Aesthet Surg 2009;62:e561)
● Causing mediastinal widening on chest Xray of morbidly obese woman (Cases J 2008 Sep 19;1:171)
● 89 year old man with familial disease (Dermatol Online J 2003;9:9)
● Familial disease with normal karyotype (Cutis 2007;79:227)

Treatment and prognosis
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● Palliative surgical removal of excess fat
● May recur
● May lead to amputation of extremity due to distortion or loss of function

Clinical images
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Familial multiple lipomatosis: lesions of abdomen and arms

Gross description
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● Poorly circumscribed aggregates of normal appearing fat in subcutaneous and skeletal muscle (but not confined to muscle)
● Does not affect nerves

Micro description
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● Sheets and lobules of white adipose tissue that may infiltrate skeletal muscle
● May involve bone

Micro images
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Lipomatosis infiltrating skeletal muscle

Positive stains
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● S100, vimentin

Additional references
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Stanford University


Adiposis dolorosa (subtopic of lipomatosis)

General
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● Tender accumulations of subcutaneous fat / lipomas, often in pelvis and lower extremities of postmenopausal women

Terminology
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● Also called Dercum’s disease

Epidemiology
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● Associated with women, obesity, hypercholesterolemia

Clinical features
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● Pain may be due to pressure of fat / lipomas on peripheral nerves

Case reports
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● 51 year old obese woman with multiple painful subcutaneous lipomas (Ann Ital Med Int 2005;20:187)
● 63 year old obese woman with recurrent abdominal pain treated medically (Dtsch Med Wochenschr 2006;131:434)

Clinical images
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Type I Dercum’s disease: generalised diffuse form with widespread painful adipose tissue but no distinct lipomas

Additional references
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eMedicine, OMIM 103200, Orphanet J Rare Dis 2012;7:23


Multiple symmetric lipomatosis (subtopic of lipomatosis)

General
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● Multiple non-encapsulated lipomas with symmetric distribution and sparing of distal arms and legs

Terminology
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● Also called Madelung’s disease, Launois-Bensaude syndrome, benign symmetric lipomatosis

Epidemiology
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● Rare, usually men, ages 40+ years
● Associated with high ethanol intake (Int J Obes Relat Metab Disord 2002;26:253)
● Women: more common in proximal arms (91%) and legs (54%); also associated with high ethanol intake (Int J Obes Relat Metab Disord 2003;27:1419)

Treatment and prognosis
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● Associated with significant morbidity, including metabolic disturbances, neuropathy, malignancy and sudden death
● Treatment - liposuction or surgery, not diet (Acta Dermatovenerol Croat 2008;16:31)
● Possibly fibrate drugs (Obes Surg 2008;18:240) or intralesional injections (Aesthetic Plast Surg 2010;34:153)

Case reports
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● 8 year old girl with bilateral neck swellings (Dentomaxillofac Radiol 2007;36:51)
● 49 year old woman post-liver transplant for cirrhosis (Dermatology 2008;216:337)

Gross description
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● Involves subcutaneous and deep soft tissue

Gross images
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Neck tumor

Clinical images
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Multiple neck masses


Various images

Micro description
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● Unencapsulated masses of mature adipose tissue

Micro images
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Resembling well differentiated liposarcoma


Adipose surrounding nerve

Molecular/cytogenetics description
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● May be associated with mitochondrial DNA abnormalities (Neurology 1994;44:862)

Additional references
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OMIM 151800

End of Soft Tissue Tumors > Adipose tissue > Lipomatosis


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