Stewart-Treves syndrome as a rare complication of a hereditary lymphedema

Vasa. 2004 Feb;33(1):42-5. doi: 10.1024/0301-1526.33.1.42.

Abstract

Lymphangiosarcoma (LAS) may occur as a rare complication of primary lymphedema. A case of LAS in hereditary lymphedema of the lower extremity in a 36-year old female is reported. Despite of chemotherapy, local hyperthermia and later amputation of the extremity the patient died of progressive disease due to pulmonary metastasis. In respect to this case, the different therapeutic concepts, as reported in the literature, and their results are presented and discussed.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Amputation, Surgical
  • Biopsy
  • Disease Progression
  • Fatal Outcome
  • Female
  • Genes, Dominant
  • Genetic Predisposition to Disease / genetics
  • Humans
  • Leg* / pathology
  • Lymphangiosarcoma / diagnosis*
  • Lymphangiosarcoma / pathology
  • Lymphangiosarcoma / surgery
  • Lymphedema / complications*
  • Lymphedema / diagnosis
  • Lymphedema / genetics*
  • Lymphedema / pathology
  • Magnetic Resonance Imaging
  • Neoplasm Invasiveness / pathology
  • Platelet Endothelial Cell Adhesion Molecule-1 / analysis
  • Skin / pathology
  • Skin Neoplasms / diagnosis*
  • Skin Neoplasms / pathology
  • Skin Neoplasms / surgery
  • Soft Tissue Neoplasms / diagnosis*
  • Soft Tissue Neoplasms / pathology
  • Soft Tissue Neoplasms / surgery
  • Syndrome

Substances

  • Platelet Endothelial Cell Adhesion Molecule-1