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Soft Tissue Tumors

Lipomatosis

 

Author: Nat Pernick, M.D., PathologyOutlines.com, Inc.

Reviewer: David Lucas, M.D., University of Michigan Health Systems (January 2009)

Revised: 26 June 2009, last major update June 2009

 

Definition

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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

● See also below - Adiposis dolorosa, Multiple symmetric lipomatosis

● See also pelvic 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 Jan 3 [Epub ahead of print])

● Causing mediastinal widening on chest Xray of morbidly obese woman (Cases J 2008 Sep 19;1(1):171)

 

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

 

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)

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Definition

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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

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

 

Case Reports

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● Obese woman with recurrent abdominal pain treated medically (Dtsch Med Wochenschr 2006;131:434)

Obese woman with multiple painful subcutaneous lipomas (Ann Ital Med Int 2005;20:187)

 

Additional references

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eMedicine, OMIM 103200

 

 

Multiple symmetric lipomatosis (subtopic of lipomatosis)

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Definition

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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

 

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)

In women is more common in proximal arms (91%) and legs (54%) and 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 2009 Jun 2 [Epub ahead of print])

 

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)

● 89 year old man with familial disease (Dermatol Online J 2003;9:9)

Familial disease with normal karyotype (Cutis 2007;79:227)

 

Clinical images

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Multiple neck masses                       Various images                                  Various images

 

Gross description

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● Involves subcutaneous and deep soft tissue

 

Gross images

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Neck tumor                         

 

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

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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 > Lipomatosis

 

 

 

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