D2-40 labeling in lymphangiomyoma/lymphangiomyomatosis of the soft tissue: further evidence of lymphangiogenic tumor histogenesis

Virchows Arch. 2007 Apr;450(4):449-53. doi: 10.1007/s00428-007-0376-9. Epub 2007 Feb 15.


We examined ten cases of extrapulmonary lymphangioleiomyoma/lymphangioleiomyomatosis (LAM; all patients female; median age 46.5 years) for immunohistochemical labeling with a monoclonal antibody against podoplanin (D2-40), which is specific for lymphatic endothelial lining. We found positive staining in thin-wall branching vessels reflecting the lymphatic nature of tumor vessels in all cases tested. In contrast, perivascular (HMB-45 positive) myoid cells were not detected by D2-40. The D2-40 labeling confirms the current concept of lymphangiogenic origin of the tumor vessels in LAM. In addition, this study makes a further contribution to the immunohistochemical mapping of this antibody in vascular tumors. Finally, the use of this commercially available antibody provides an additional help in the differential diagnosis of LAM from other soft tissue tumors.

MeSH terms

  • Actins / analysis
  • Adult
  • Aged
  • Antibodies, Monoclonal / analysis*
  • Antibodies, Monoclonal / immunology
  • Antigens, Neoplasm
  • Female
  • Humans
  • Immunohistochemistry
  • Lymphangiogenesis
  • Lymphangioleiomyomatosis / metabolism
  • Lymphangioleiomyomatosis / pathology
  • Lymphangiomyoma / metabolism
  • Lymphangiomyoma / pathology*
  • Lymphatic Vessels / chemistry
  • Lymphatic Vessels / pathology
  • Melanoma-Specific Antigens
  • Membrane Glycoproteins / analysis*
  • Membrane Glycoproteins / immunology
  • Middle Aged
  • Muscle, Smooth / chemistry
  • Neoplasm Proteins / analysis
  • Platelet Endothelial Cell Adhesion Molecule-1 / analysis
  • Vascular Neoplasms / metabolism
  • Vascular Neoplasms / pathology*


  • Actins
  • Antibodies, Monoclonal
  • Antigens, Neoplasm
  • Melanoma-Specific Antigens
  • Membrane Glycoproteins
  • Neoplasm Proteins
  • PDPN protein, human
  • Platelet Endothelial Cell Adhesion Molecule-1