title: Primary angiosarcoma of bone: a retrospective analysis of 60 patients from 2 institutions.
abstract: BACKGROUND
  Angiosarcoma of bone is a rare high-grade malignant vascular tumor. The literature regarding treatment and outcome of patients with this tumor is limited.We performed a 2 institutional retrospective study to analyze treatment and survival of patients with angiosarcoma of bone.
  
  
  METHODS
  We reviewed patients with the histologic diagnosis of primary angiosarcoma of bone treated from 1980 to 2009. Demographic details, histology, treatment, and survival were reviewed.
  
  
  RESULTS
  A total of 38 men and 22 women (median age, 54 y) were recruited. Most lesions occurred in the femur and the pelvis. Metastatic disease at presentation was diagnosed in 24 patients (40%). Forty-three patients underwent surgery, with 30 of them achieving surgical complete remission (SCR). Radiotherapy was applied to 17 patients, and chemotherapy to 13/35 and 15/22 patients with localized and metastatic disease, respectively.The 5-year overall survival (OS) was 20%: 33% for patients with localized disease and 0% for metastatic patients. Higher 5-year OS was reported for patients who achieved SCR (46%) than for those who did not (0%). In nonmetastatic patients, a trend toward improved survival was observed after SCR and adjuvant chemotherapy based on cisplatin, doxorubicin, and ifosfamide.Fifteen patients received chemotherapy for metastases. Two RECIST partial responses of 13 evaluable patients were documented (paclitaxel [n=1] and doxorubicin [n=1]). Stable disease was observed in 2 patients.
  
  
  CONCLUSIONS
  Complete surgical resection is essential for outcome. Survival of patients with metastatic or unresectable disease is very poor. Activity of taxanes and anthracycline was observed in the metastatic setting and merits further evaluation.
authors: Departments of *Chemotherapy ‡Orthopaedic Surgery §Surgical Pathology ¶Research Laboratory, Musculoskeletal Oncology Department, Istituto Ortopedico Rizzoli, Bologna, Italy Departments of †Medicine ∥Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY.
authors: Emanuela
authors: E
authors: Palmerini
authors: Robert G
authors: RG
authors: Maki
authors: Eric L
authors: EL
authors: Staals
authors: Marco
authors: M
authors: Alberghini
authors: Cristina R
authors: CR
authors: Antonescu
authors: Crisitina
authors: C
authors: Ferrari
authors: Pietro
authors: P
authors: Ruggieri
authors: Andreas
authors: A
authors: Mavrogenis
authors: Franco
authors: F
authors: Bertoni
authors: Marilena
authors: M
authors: Cesari
authors: Anna
authors: A
authors: Paioli
authors: Emanuela
authors: E
authors: Marchesi
authors: Piero
authors: P
authors: Picci
authors: Stefano
authors: S
authors: Ferrari
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000473:pathology / Q000628:therapy*; D002648:Child; D002945:Cisplatin / Q000008:administration & dosage; D015331:Cohort Studies; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D005266:Femoral Neoplasms / Q000473:pathology / Q000628:therapy; D005269:Femur / Q000473:pathology / Q000601:surgery; D006394:Hemangiosarcoma / Q000473:pathology / Q000628:therapy*; D006801:Humans; D006811:Humerus / Q000473:pathology / Q000601:surgery; D007069:Ifosfamide / Q000008:administration & dosage; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D008875:Middle Aged; D020360:Neoadjuvant Therapy; D017239:Paclitaxel / Q000008:administration & dosage; D010384:Pelvic Bones / Q000473:pathology / Q000601:surgery; D011878:Radiotherapy; D018714:Radiotherapy, Adjuvant; D011884:Radius / Q000473:pathology / Q000601:surgery; D012189:Retrospective Studies; D012888:Skull Neoplasms / Q000473:pathology / Q000601:surgery; D013125:Spinal Neoplasms / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome; D014457:Ulna / Q000473:pathology / Q000601:surgery; D055815:Young Adult
chemical_list: D004317:Doxorubicin; D017239:Paclitaxel; D002945:Cisplatin; D007069:Ifosfamide; D008727:Methotrexate