title: Angiosarcoma (Stewart-Treves syndrome) in postmastectomy patients: report of 10 cases and review of literature.
abstract: OBJECTIVE
  To study the clinicopathologic features of Stewart-Treves syndrome (STS) in postmastectomy patients including the epidemiology, presentation, morphology, differentiation, pathogenesis and therapeutic options.
  
  
  RESULTS
  Ten cases of STS in postmastectomy patients were retrospectively identified in our archives, and immunohistochemistry for CD34, CD31, D2-40, HHV-8, CK, EMA and Ki-67 was performed. All ten patients presented with lymphedema after mastectomy as the first sign. Physical examination revealed multiple raised, pinkish-red papulo-vesicular lesions or ulceration as the early evidence of tumor in the field where radiation therapy was introduced. Microscopic examination revealed infiltrative proliferation of vessels and the heteromorphic tumor cells expressed CD34, CD31 and D2-40. Despite the various treatment modalities, 5 patients died in an average of 19 months, 4 patients survived to the last follow-up (9-31 months), and 1 patient got lost.
  
  
  CONCLUSIONS
  STS is a fatal complication of postmastectomy lymphedema. Patients with STS have very poor prognosis. The key to improve patient's survival is the early diagnosis through a high alert of this disease by primary care physicians and comprehensive physical examination of patients with pertinent history and suspicious clinical presentations followed by prompt biopsy for definitive diagnosis.
authors.affiliation: Department of Pathology, Beijing Shijitan Hospital, Capital Medical University China.
authors.forename: Lifang
authors.initials: L
authors.lastname: Cui
authors.affiliation: Department of Pathology, Peking University First Hospital Beijing, China.
authors.forename: Jixin
authors.initials: J
authors.lastname: Zhang
authors.affiliation: Department of Pathology and Laboratory Medicine, Temple University Hospital Philadelphia, PA, USA.
authors.forename: Xinmin
authors.initials: X
authors.lastname: Zhang
authors.affiliation: Department of Pathology, Beijing Shijitan Hospital, Capital Medical University China.
authors.forename: Hong
authors.initials: H
authors.lastname: Chang
authors.affiliation: Department of Pathology, Beijing Shijitan Hospital, Capital Medical University China.
authors.forename: Congling
authors.initials: C
authors.lastname: Qu
authors.affiliation: Department of Pathology, Beijing Shijitan Hospital, Capital Medical University China.
authors.forename: Jiangying
authors.initials: J
authors.lastname: Zhang
authors.affiliation: Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College Beijing 100730, China.
authors.forename: Dingrong
authors.initials: D
authors.lastname: Zhong
mesh_terms: D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D001943:Breast Neoplasms / Q000532:radiotherapy* / Q000601:surgery*; D055088:Early Detection of Cancer; D017809:Fatal Outcome; D005260:Female; D006394:Hemangiosarcoma / Q000737:chemistry / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D008204:Lymphangiosarcoma / Q000737:chemistry / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D008408:Mastectomy / Q000009:adverse effects*; D008875:Middle Aged; D009381:Neoplasms, Radiation-Induced / Q000737:chemistry / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D011237:Predictive Value of Tests; D018714:Radiotherapy, Adjuvant / Q000009:adverse effects; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor
keywords: Angiosarcoma; breast cancer; extremity swelling; radiation