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Telangiectatic osteosarcoma: a review of 87 cases

  • Original Article – Clinical Oncology
  • Published:
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Abstract

Purposes

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.

Level of evidence

IV.

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Acknowledgments

We wish to thank our pathologist Marco Gambarotti, MD for their contribution to the review of all histopathologic slides and our epidemiologist Cristina Ferrari for her help in the collection of data and epidemiologic review. This paper is dedicated to the memory of our beloved mentor and friend Prof. Mario Mercuri that we severely miss.

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Authors and Affiliations

  1. Department of Orthopedics, Istituto Ortopedico Rizzoli, University of Bologna, Bologna, Italy

    Andrea Angelini, Andreas F. Mavrogenis & Giulia Trovarelli

  2. Department of Chemotherapy, Istituto Ortopedico Rizzoli, Bologna, Italy

    Stefano Ferrari

  3. Department of Oncology, Istituto Ortopedico Rizzoli, Bologna, Italy

    Piero Picci

  4. Department of Orthopedics and Orthopedic Oncology, University of Padova, Via Giustiniani, Padova, Italy

    Pietro Ruggieri

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  1. Andrea Angelini
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  2. Andreas F. Mavrogenis
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  3. Giulia Trovarelli
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  4. Stefano Ferrari
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  5. Piero Picci
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  6. Pietro Ruggieri
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Correspondence to Pietro Ruggieri.

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Angelini, A., Mavrogenis, A.F., Trovarelli, G. et al. Telangiectatic osteosarcoma: a review of 87 cases. J Cancer Res Clin Oncol 142, 2197–2207 (2016). https://doi.org/10.1007/s00432-016-2210-8

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