Lipomatosis
Definition
- Diffuse or regional overgrowth of fat
Diagnostic Criteria
- Diffuse or regional overgrowth of mature fat
- May be difficult to distinguish from multiple discrete lipomas
- May infiltrate adjacent skeletal muscle
- Diffuse lipomatosis
- Diffuse overgrowth of mature fat
- Usually involves a large region of an extremity or trunk
- Age usually under 2 years
- Involves muscle and subcutaneous tissue
- Does not involve nerves
- Recurs frequently
- Encephalocraniocutaneous lipomatosis
- Haberland syndrome
- Lipomatous hamartomas of the head and face
- Usually unilateral
- Congenital
- Cranial asymmetry and mental retardation
- Fibrolipomatous hamartoma of nerve / neural fibrolipoma / lipomatosis of nerve
- Mature adipose tissue with a fibrous component
- Epineural and perineural fibrosis
- Metaplastic bone has been reported
- Surrounds and infiltrates a segment of a major nerve
- Most often involves median nerve
- May have associated bone overgrowth causing macrodactyly
- Usually under age 30
- Usually causes neural signs and symptoms
- Benign but may require excision or release of compression
- Mature adipose tissue with a fibrous component
- Lumbosacral lipoma / lipomatosis
- Diffuse increase in mature fat over lumbosacral spine
- Spina bifida or laminar defect always present
- Meningocele or myelocele may be present
- Usually in infants and children
- May have associated skin tags, hemangioma or hair overgrowth
- Pelvic lipomatosis
- Perivesical and perirectal overgrowth
- Rarely involves retroperitoneum and mesentery
- Frequent cystitis glandularis
- May develop adenocarcinoma
- Usually in black males
- Age 9-80
- Perivesical and perirectal overgrowth
- Symmetrical lipomatosis
- Madelung or Launois-Bensaude syndrome
- Usually neck, shoulders, upper arms
- Described as pseudoathletic or horse collar
- Frequently associated with diabetes and alcohol abuse
- More common in men of Mediterranean origin
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting : July 29, 2007
