Article Contributors: 
    Sean Klepper M.D.
    Stephen Lyle, M.D., ...

    Clinical Features:

    • Proliferation of lymphatic vessels with dilatation, probably hamartomatous in origin
    • Most are congenital or present within the first years of life.
    • Several clinical types exist, although sometimes overlap makes precise classification difficult:
      • Lymphangioma simplex: Solitary, well-circumscribed, skin-colored lesion presenting in infancy
      • Lymphangioma circumscriptum: Most common type.  Presents as cutaneous vesicles.  Similar lesions are sometimes seen secondary to impaired lymphatic drainage: status post radical mastectomy or radiation therapy as well as vulvar lesions that arise due to impaired lymphatic drainage from a variety of causes.
        • The localized form presents as a single small lesion at any age.
        • The classic form presents at birth through early childhood.  It is larger and may be single or multiple and may be extensive.
      • Cavernous lymphangioma: Presents at birth or infancy.  Large, poorly defined lesion, most commonly involving the head, neck, mouth or extremities.
        • Cystic hygroma: Type of cavernous lymphangioma with large, deforming cystic lymphatic dilatation.  Seen on the neck in patients with Turner syndrome.  Other sites include the axilla, groin, popliteal fossa, mediastinum and retroperitoneum.

    Histologic Features:

    • All types of lymphangiomas show proliferations of lymphatic channels lined by flat endothelial cells.  Their lumina contain homogenous, pale-staining fluid and sometimes some red blood cells secondary to hemorrhage. 
    • Lymphagioma simplex: Proliferation of capillary-sized lymphatics
    • Lymphangioma circumscriptum:
      • Dilated lymphatics in the upper dermis
      • The overlying epidermis is often elevated and ranges from thin to acanthotic.
      • Papillomatosis and hyperkeratosis may be present, especially in the classic type.
      • The classic type extends deeper into the dermis and even subcutis, whereas the localized type is more superficial.
    • Cavernous lymphangioma:
      • Widely dilated lymphatics expanding the dermis and subcutis
      • There is sometimes fibrotic stroma separating the lymphatic channels.
      • The lymphatic walls may contain smooth muscle.
    • Cystic hygroma:
      • Large unilocular or multilocular thin-walled cysts
      • The lymphatic walls may contain smooth muscle.

    Cases associated with this book:

  • Lymphangioma
    Author: Stephen Lyle, M.D., Ph.D.

    Conference: Teaching Collection