Pathology: lipomatosis


Clinical history of congenital lipomatosis

  • Adipocytes arranged in sheets

  • Abundant clear cytoplasm with minimal variability in size and shape

  • Peripherally located small monomorphic nuclei

  • Entrapped skeletal muscle fibers may show atrophy, which should not be confused with atypia of liposarcoma

  • FISH: negative for MDM2 amplification

  • Associated with mitochondrial DNA mutations (PMID: 9309699, 31912494)