Lipoblastoma

DEFINITION AND PATHOGENESIS

IMPORTANCE

  • Occurs in infancy and early childhood, confined to the subcutaneous tissue (has been reported in mediastinum, mesentery, and retroperitoneum as well)

CLINICAL FEATURES

RADIOLOGIC FEATURES

GROSS PATHOLOGY

HISTOLOGIC FEATURES

  • Lipoblasts in various stages of development (spindle-shaped mesenchymal cells to almost "signet ring" of lipocyte)
  • Very rare mitoses if at all
  • Histologic variations
    • Subtle zonal architecture of fat maturation
    • Abundant myxoid material
    • Primitive mesenchymal cells
    • Focal plexiform vascular pattern
    • Multinucleated cells
  • Immunohistochemistry
  • Cytogenetics
    • Chromosomal 8 abnormalities (8/59
      • A case with seven copies of chromosome 8 has been reported

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Myxoid liposarcoma
  • Lipoma
  • Hibernoma

DISEASE COURSE AND TREATMENT

  • Rarely recurs after wide local resection, 9-46%% LR reported
    • The degreee of adipocytic differentiation does not predict biologic behavior
      • In the head and neck location, LR is reported to be 27
        • Complete surgical resection often arduous in head/neck
        • Resolve of respiratory distress often the first goal in infants
      • Periodic follow up necessary for many yrs
  • Unplanned excisions (excision before proper investigations) may result in incomplete resection, LR, and further potentially mutilating surgery

SPECIAL CONSIDERATIONS

  • Lipoblastomatosis

    • The diffuse form of lipoblastoma
      • ("Infiltrating lipoblastoma")
      • Extends across anatomic planes and into different tissues
    • M:F = 2:1
    • Diffusely involves skin and muscle in children
    • Partial resection of an intraspinal cervical lipoblastoma in infancy has been reported to recur as a lipoma in a 4-yr-old girl
    • Greater tendency to recur


  • Lipoblastoma Associated with Congenital Anomalies

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