Lipoblastoma and Lipoblastomatosis
Amitabh Srivastava, MD
Key Facts
Terminology
Benign tumor resembling fetal adipose tissue
Lipoblastomas are well circumscribed
Lipoblastomatosis is deeply infiltrative
Clinical Issues
Presents in infancy and early childhood
Macroscopic Features
Pale, lobulated, and myxoid appearance
Microscopic Pathology
Lobules of immature adipocytes separated by fibrous septa
Myxoid stroma with plexiform vasculature resembling myxoid liposarcoma
Ancillary Tests
Rearrangement of 8q11-13 most common cytogenetic anomaly
 Lipoblastomas have a glistening, pale yellow, lobular appearance with interlobular fibrous septation  , which may also be apparent on gross examination. |
 Lipoblastomas are characterized on low power by variably thick fibrous septa  that impart a lobular configuration to the tumor. |
TERMINOLOGY
Synonyms
Fetal lipoma
Infantile lipoma
Embryonic lipoma
Definitions
Benign tumor resembling fetal adipose tissue
Localized tumors designated lipoblastoma
Diffuse tumors designated lipoblastomatosis
CLINICAL ISSUES
Epidemiology
Incidence
Rare tumors
Age
Most common in 1st 3 years of life
Rare in older children
Gender
More common in males
Site
Extremities are most common site of involvement
May involve head/neck or trunk, mediastinum, retroperitoneum, or even visceral sites
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