Telangiectatic osteosarcoma: a review of 87 cases

Andrea Angelini, Andreas F. Mavrogenis, Giulia Trovarelli, Stefano Ferrari, Piero Picci, Pietro Ruggieri

Research output: Contribution to journalArticle

12 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)1-11
Number of pages11
JournalJournal of Cancer Research and Clinical Oncology
DOIs
Publication statusAccepted/In press - Jul 28 2016

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Osteosarcoma
Neoplasm Metastasis
Drug Therapy
Spontaneous Fractures
Tumor Burden
Survival
Necrosis
Recurrence
Lung
Limb Salvage
Amputation
Multivariate Analysis
Extremities
Bone and Bones
Therapeutics
Neoplasms

Keywords

  • Bone sarcoma
  • Chemotherapy
  • Pathologic fracture
  • Prognostic factors
  • Statistical analysis

ASJC Scopus subject areas

  • Medicine(all)
  • Oncology
  • Cancer Research

Cite this

Angelini, A., Mavrogenis, A. F., Trovarelli, G., Ferrari, S., Picci, P., & Ruggieri, P. (Accepted/In press). Telangiectatic osteosarcoma: a review of 87 cases. Journal of Cancer Research and Clinical Oncology, 1-11. https://doi.org/10.1007/s00432-016-2210-8

Telangiectatic osteosarcoma : a review of 87 cases. / Angelini, Andrea; Mavrogenis, Andreas F.; Trovarelli, Giulia; Ferrari, Stefano; Picci, Piero; Ruggieri, Pietro.

In: Journal of Cancer Research and Clinical Oncology, 28.07.2016, p. 1-11.

Research output: Contribution to journalArticle

Angelini, A, Mavrogenis, AF, Trovarelli, G, Ferrari, S, Picci, P & Ruggieri, P 2016, 'Telangiectatic osteosarcoma: a review of 87 cases', Journal of Cancer Research and Clinical Oncology, pp. 1-11. https://doi.org/10.1007/s00432-016-2210-8
Angelini A, Mavrogenis AF, Trovarelli G, Ferrari S, Picci P, Ruggieri P. Telangiectatic osteosarcoma: a review of 87 cases. Journal of Cancer Research and Clinical Oncology. 2016 Jul 28;1-11. https://doi.org/10.1007/s00432-016-2210-8
Angelini, Andrea ; Mavrogenis, Andreas F. ; Trovarelli, Giulia ; Ferrari, Stefano ; Picci, Piero ; Ruggieri, Pietro. / Telangiectatic osteosarcoma : a review of 87 cases. In: Journal of Cancer Research and Clinical Oncology. 2016 ; pp. 1-11.
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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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N2 - 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.

AB - 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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