title: Telangiectatic osteosarcoma: a review of 87 cases.
abstract: OBJECTIVE
  Telangiectatic osteosarcoma (TOS) is a rare subtype of osteosarcoma. We analyzed (1) oncologic outcome in a large homogeneous series and (2) the role of prognostic factors on prognosis, local recurrence and metastasis.
  
  
  METHODS
  Eighty-seven patients (47 males, 54 %) were retrospectively analyzed. All except 4 had extracompartmental disease, and ten patients had lung metastasis at diagnosis. Pathologic fracture was present in 27 cases (31 %). Seventy-eight patients were treated with neoadjuvant chemotherapy; nine had surgery as first treatment. Limb-salvage surgery was performed in 71 cases, amputation in 14, and rotationplasty in one. One patient died before surgery. Possible prognostic factors were statistically evaluated.
  
  
  RESULTS
  Overall survival was 60.7 % at 10 years of follow-up. Fifty-one patients were disease-free (58.6 %), 2 were alive with disease (2.3 %), 31 died with disease (35.6 %), and 3 died of other causes (3.4 %). Ten local recurrences were observed (11 %). Twenty-five patients (29 %) developed lung (22) or bone (3) metastases. No statistical difference was found considering age, metastases at diagnosis, gender, pathologic fracture, tumor volume, compartmental status, number of neoadjuvant chemotherapy agents and treatment. Induced necrosis was significant at both univariate and multivariate analysis (p < 0.0001).
  
  
  CONCLUSIONS
  TOS does not have a poor prognosis as previously reported in literature, with a survival of about 60 % at 10 years. Most of patients can be cured with neoadjuvant chemotherapy plus surgery (limb sparing surgery is possible and safe). Tumor response to chemotherapy as induced necrosis was the only significant prognostic factors on survival, even if small tumor volume at diagnosis correlates with better prognosis at univariate analysis.
  
  
  METHODS
  IV.
authors.affiliation: Department of Orthopedics, Istituto Ortopedico Rizzoli, University of Bologna, Bologna, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Angelini
authors.affiliation: Department of Orthopedics, Istituto Ortopedico Rizzoli, University of Bologna, Bologna, Italy.
authors.forename: Andreas F
authors.initials: AF
authors.lastname: Mavrogenis
authors.affiliation: Department of Orthopedics, Istituto Ortopedico Rizzoli, University of Bologna, Bologna, Italy.
authors.forename: Giulia
authors.initials: G
authors.lastname: Trovarelli
authors.affiliation: Department of Chemotherapy, Istituto Ortopedico Rizzoli, Bologna, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Ferrari
authors.affiliation: Department of Oncology, Istituto Ortopedico Rizzoli, Bologna, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Picci
authors.affiliation: Department of Orthopedics and Orthopedic Oncology, University of Padova, Via Giustiniani, Padova, Italy. pietro.ruggieri@unipd.it.
authors.forename: Pietro
authors.initials: P
authors.lastname: Ruggieri
mesh_terms: D000293:Adolescent; D000328:Adult; D001859:Bone Neoplasms / Q000473:pathology* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D012516:Osteosarcoma / Q000473:pathology* / Q000628:therapy*; D011379:Prognosis; D016032:Randomized Controlled Trials as Topic; D012189:Retrospective Studies; D055815:Young Adult
keywords: Bone sarcoma; Chemotherapy; Pathologic fracture; Prognostic factors; Statistical analysis