Home Chapter Home Jobs Conferences Fellowships Books
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
=========================================================================
● Diffuse overgrowth of mature adipose tissue
Epidemiology
=========================================================================
● 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
=========================================================================
● Limb (may cause massive enlargement) or trunk
Case reports
=========================================================================
● 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
=========================================================================
● Palliative surgical removal of excess fat
● May recur
● May lead to amputation of extremity due to distortion or loss of function
Gross description
=========================================================================
● Poorly circumscribed aggregates of normal appearing fat in subcutaneous and skeletal muscle (but not confined to muscle)
● Does not affect nerves
Micro description
=========================================================================
● Sheets and lobules of white adipose tissue that may infiltrate skeletal muscle
● May involve bone
Micro images
=========================================================================
Lipomatosis infiltrating skeletal muscle
Positive stains
=========================================================================
● S100, vimentin
Additional references
=========================================================================
Adiposis dolorosa (subtopic of lipomatosis)
Definition
=========================================================================
● Tender accumulations of subcutaneous fat / lipomas, often in pelvis and lower extremities of postmenopausal women
Terminology
=========================================================================
● Also called Dercum’s disease
Epidemiology
=========================================================================
● Associated with women, obesity, hypercholesterolemia
Clinical
=========================================================================
● Pain may be due to pressure of fat / lipomas on peripheral nerves
Case Reports
=========================================================================
● 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
=========================================================================
Multiple symmetric lipomatosis (subtopic of lipomatosis)
Definition
=========================================================================
● Multiple non-encapsulated lipomas with symmetric distribution and sparing of distal arms and legs
Terminology
=========================================================================
● Also called Madelung’s disease, Launois-Bensaude syndrome
Epidemiology
=========================================================================
● 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
=========================================================================
● 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
=========================================================================
● 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
=========================================================================
Multiple neck masses Various images Various images
Gross description
=========================================================================
● Involves subcutaneous and deep soft tissue
Gross images
=========================================================================
Neck tumor
Micro description
=========================================================================
● Unencapsulated masses of mature adipose tissue
Micro images
=========================================================================
Resembling well differentiated liposarcoma
Adipose surrounding nerve
Molecular / cytogenetics
=========================================================================
● May be associated with mitochondrial DNA abnormalities (Neurology 1994;44:862)
Additional references
=========================================================================
End of Soft Tissue Tumors > Lipomatosis
This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must also be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.
All information on this website is protected by Copyright, (c) 2001-2009, PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions.