Clinical epidemiology and treatment outcomes of spindle cell non-osteogenic bone sarcomas – A nationwide population-based study

Kjetil Berner, Tom Børge Johannesen, Kirsten Sundby Hall, Oyvind Bruland

Research output: Contribution to journalArticle

Abstract

Purpose: To describe epidemiological and clinical characteristics, as well as long-term treatment outcomes of spindle cell non-osteogenic bone sarcomas (SCS), comprising leiomyosarcoma, fibrosarcoma and undifferentiated pleomorphic sarcoma in bone. Method: We have analysed a nationwide cohort of 104 patients with histologically verified SCS diagnosed between 1975 and 2009, based on registry sources supplemented with clinical records from Norwegian hospitals involved in sarcoma management. Results: In this unselected cohort, a stable annual incidence for SCS patients of slightly below 0.6 per million was observed, with a dominant peak among elderly patients. SCS is mostly a high-grade malignancy (92%) with a male to female ratio of 1.6 for all patients. The axial to appendicular ratio was 0.7, seemingly independent of age. More than one fourth of the patients (29%) had primary metastatic disease. Another 32 patients (46%) developed metastases during follow-up and 12 (17%) experienced local relapses. The five-year sarcoma-specific survival rate was 37%, with no documented improvement over time. Primary metastatic disease was an adverse prognostic factor for survival. Predisposing factors were documented in 19 patients (18%). Negative prognostic factors for overall survival were tumour size >9 cm, age > 40 years, axial tumour localization, FS as subtype and pathologic fracture at time of diagnoses. As expected, patients who received both surgery and chemotherapy as their primary treatment for high-grade SCS (25%) significantly had best sarcoma specific five years survival (62%). Conclusion: We confirm SCS as a rare high-grade bone sarcoma entity, mostly among elderly patients and with a poor overall outcome. The combined treatment of surgery and chemotherapy is essential to achieve optimal long-term survival of SCS.

LanguageEnglish (US)
Article number100207
JournalJournal of Bone Oncology
Volume14
DOIs
StatePublished - Feb 1 2019

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Sarcoma
Epidemiology
Bone and Bones
Population
Survival
Drug Therapy
Neoplasms
Spontaneous Fractures
Leiomyosarcoma
Hospital Records
Fibrosarcoma
Causality
Registries
Survival Rate
Neoplasm Metastasis
Recurrence

Keywords

  • Nationwide
  • Overall survival
  • Spindle cell
  • Treatment

ASJC Scopus subject areas

  • Oncology

Cite this

Clinical epidemiology and treatment outcomes of spindle cell non-osteogenic bone sarcomas – A nationwide population-based study. / Berner, Kjetil; Johannesen, Tom Børge; Hall, Kirsten Sundby; Bruland, Oyvind.

In: Journal of Bone Oncology, Vol. 14, 100207, 01.02.2019.

Research output: Contribution to journalArticle

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abstract = "Purpose: To describe epidemiological and clinical characteristics, as well as long-term treatment outcomes of spindle cell non-osteogenic bone sarcomas (SCS), comprising leiomyosarcoma, fibrosarcoma and undifferentiated pleomorphic sarcoma in bone. Method: We have analysed a nationwide cohort of 104 patients with histologically verified SCS diagnosed between 1975 and 2009, based on registry sources supplemented with clinical records from Norwegian hospitals involved in sarcoma management. Results: In this unselected cohort, a stable annual incidence for SCS patients of slightly below 0.6 per million was observed, with a dominant peak among elderly patients. SCS is mostly a high-grade malignancy (92{\%}) with a male to female ratio of 1.6 for all patients. The axial to appendicular ratio was 0.7, seemingly independent of age. More than one fourth of the patients (29{\%}) had primary metastatic disease. Another 32 patients (46{\%}) developed metastases during follow-up and 12 (17{\%}) experienced local relapses. The five-year sarcoma-specific survival rate was 37{\%}, with no documented improvement over time. Primary metastatic disease was an adverse prognostic factor for survival. Predisposing factors were documented in 19 patients (18{\%}). Negative prognostic factors for overall survival were tumour size >9 cm, age > 40 years, axial tumour localization, FS as subtype and pathologic fracture at time of diagnoses. As expected, patients who received both surgery and chemotherapy as their primary treatment for high-grade SCS (25{\%}) significantly had best sarcoma specific five years survival (62{\%}). Conclusion: We confirm SCS as a rare high-grade bone sarcoma entity, mostly among elderly patients and with a poor overall outcome. The combined treatment of surgery and chemotherapy is essential to achieve optimal long-term survival of SCS.",
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