title: Lymphangiosarcoma: Is Stewart-Treves Syndrome a Preventable Condition?
abstract: BACKGROUND
  Stewart-Treves syndrome is a rare complication of breast cancer treatment, representing a lymphangiosarcoma commonly associated with lymphedema and severely impacting patient's outcome. The tumor typically develops in the atrophic, pachydermatous, hyperkeratotic skin of limbs affected by long-standing lymphedema. Clinical data associated with Stewart-Treves syndrome and lymphedema management have rarely been published.
  
  
  RESULTS
  In the period between 1980 and 2009, ten patients with Stewart-Treves syndrome were diagnosed and treated at the Foeldiklinik, Hinterzarten, Germany. Nine of the ten patients were female. Five patients had previously suffered from breast cancer (and were treated with mastectomy); two from other malignancies; two patients had primary lymphedema, and one had undergone lower extremity lymphadenectomy. All cancer patients had undergone radiation treatment. In all cases, the sarcoma developed in non-irradiated areas 6-48 years (average 16.3 years) after the onset of lymphedema. None of the patients had received complex decongestive physical therapy (CDT). Two patients had above-elbow amputation, one had shoulder exarticulation, two patients had wide excision and skin grafting, two patients had above-knee amputation procedure, two patients had a below-knee amputation procedure, and one patient had no surgical treatment at all. The time to recurrence after surgery, time to metastasis, patient survival and CDT were recorded.
  
  
  CONCLUSIONS
  Patients with lymphedema should be closely examined starting 5 years from the time of lymphedema onset, paying special attention to those with associated malignancies. Only early diagnosis and treatment by radical ablative surgery confers a reasonable prognosis with this rare but aggressive disease. A potential effect of CDT on lymphangiosarcoma has to be studied in a greater patient cohort.
authors.affiliation: 1 Division of Plastic Surgery, Department of Trauma Surgery, Plastic and Reconstructive Surgery, University Medical Centre , Göttingen, Germany .
authors.forename: Gunther
authors.initials: G
authors.lastname: Felmerer
authors.affiliation: 2 Division of Plastic Surgery, University of Massachusetts Medical School , Worcester, Massachusetts.|3 Department of Plastic and Hand Surgery, University of Freiburg Medical Center , Freiburg, Germany .
authors.forename: A S
authors.initials: AS
authors.lastname: Dowlatshahi
authors.affiliation: 3 Department of Plastic and Hand Surgery, University of Freiburg Medical Center , Freiburg, Germany .
authors.forename: G Bjoern
authors.initials: GB
authors.lastname: Stark
authors.affiliation: 4 Lymphologische Fachklinik , Földiklinik, Hinterzarten, Germany, Competence Network for Lymphology, Freiburg-Hinterzarten, Germany .
authors.forename: Ethelka
authors.initials: E
authors.lastname: Földi
authors.affiliation: 4 Lymphologische Fachklinik , Földiklinik, Hinterzarten, Germany, Competence Network for Lymphology, Freiburg-Hinterzarten, Germany .
authors.forename: Martha
authors.initials: M
authors.lastname: Földi
authors.affiliation: 5 Institute of Pathology, University Medical Centre , Göttingen, Germany .
authors.forename: Maria G
authors.initials: MG
authors.lastname: Ahls
authors.affiliation: 5 Institute of Pathology, University Medical Centre , Göttingen, Germany .
authors.forename: Philipp
authors.initials: P
authors.lastname: Ströbel
authors.affiliation: 1 Division of Plastic Surgery, Department of Trauma Surgery, Plastic and Reconstructive Surgery, University Medical Centre , Göttingen, Germany .
authors.forename: Thiha
authors.initials: T
authors.lastname: Aung
mesh_terms: D001943:Breast Neoplasms / Q000150:complications; D005260:Female; D006394:Hemangiosarcoma / Q000150:complications / Q000601:surgery*; D006801:Humans; D008204:Lymphangiosarcoma / Q000150:complications / Q000601:surgery*; D008209:Lymphedema / Q000150:complications; D008297:Male; D011471:Prostatic Neoplasms / Q000150:complications; D016019:Survival Analysis; D016896:Treatment Outcome